CHAPTER 11

Hematology-Oncology

247
 248 Section II: Organ Systems • Questions

Q U E ST I O N S

1. A 23-year-old woman presents to her physician (C) Intrinsic factor

with a nevus on her arm that is asymmetrical (D) Secretin
with irregular borders. She has noticed a (E) Somatostatin
change in the color of this nevus in recent
weeks. The nevus is removed and sent to the 4. A 65-year-old man presents with a 2-month his-
pathologist. On examination of the specimen, tory of a cough. He also complains of a droopy
the pathologist determines that it is a mela- right eyelid and dry facial skin. On a head and
noma. Which of the following features of this neck examination, the physician finds the right
lesion is the greatest indicator of a poor prog- pupil is more constricted than the left one. CT
nosis for this patient? scan reveals a 3-cm nodule in one lung. Based
HIGH-YIELD SYSTEMS

on these symptoms, which of the following is

(A) Depth within the dermis of >2 mm
the most likely location of the tumor found on
(B) Growth location on her arm
CT scan?
(C) Lateral spread within the epidermis of
>5 mm (A) Apex of left upper lobe
(D) Previous basal cell carcinoma lesions (B) Apex of right upper lobe
(E) Very large average size of neoplastic cells (C) Hilum
(D) Right lower lobe
2. A 57-year-old smoker presents to her primary (E) Right middle lobe
care physician with a lump in the upper outer
quadrant of her right breast. Mammography 5. A 58-year-old woman presented to her physi-
confirms a 2.5-cm lesion and core needle bi- cian after discovering a lump in her left breast.
opsy yields invasive ductal carcinoma. The pa- The physician examines the lump and finds it
tient undergoes lumpectomy with axillary to be hard, nontender, and moveable. Which
lymph node dissection, and is referred to an of the following increases this woman’s risk of
Hematology-Oncology

oncologist to begin chemotherapy. Which of breast cancer?

the following should be coadministered with (A) Drinking 4 cups of coffee every day
the chemotherapy that this patient will most (B) Having a history of a cyst that drained
likely receive? straw-colored fluid
(A) Leucovorin (C) Having a history of fibroadenoma of the
(B) Mesna left breast
(C) Thymidine (D) Having gone through menopause 2 years
(D) Vitamin B12 ago
(E) Vitamin B6 (E) Taking St. John’s wort every day

3. A 43-year-old man comes to the physician be- 6. Low-molecular-weight heparins (LMWH) are
cause of increasing epigastric pain, heartburn, distinct from unfractionated heparin in several
and weight loss over the past 4 months. When ways. Which of the following is the primary
asked about bowel habits, the patient recalls a target of LMWH?
few occasions on which he had black, tarry (A) Antithrombin III
stools. On physical examination, the physician (B) Factor IIa
notes that the patient is thin and pale and has (C) Factor VII
diffuse tenderness over the epigastric area. Se- (D) Factor Xa
rum levels of which of the following substances (E) Factors II, IX, and X
are likely to be elevated in this patient?
(A) Cholecystokinin 7. A 4-year-old girl is brought to the emergency
(B) Gastrin department with an 8-hour history of projectile
 Chapter 11: Hematology-Oncology • Questions 249

vomiting and headache. Her parents say that patient does not have tremor, restlessness, heat
the patient was well until 2 months ago, when intolerance, or an increased level of anxiety.
they noted that she was becoming increasingly Blood tests show normal thyroid hormone and
clumsy. Physical examination shows nystagmus calcitonin levels. A scintiscan shows a cold
in all directions of gaze, as well as truncal nodule in the left lobe of her thyroid. Tissue is
ataxia. Laboratory studies of blood show a obtained and a histological section is shown in
WBC count of 7200/mm³, a hemoglobin level the image. This patient most likely has which
of 12.3 g/dL, and a platelet count of 225,000/ of the following?
mm³. A CT scan shows a lesion in the cerebel-
lar vermis with associated dilation of the third
and lateral ventricles. Which of the following
is most likely to be evident on histopathologic

HIGH-YIELD SYSTEMS
examination of the lesion?
(A) Deeply staining nuclei with scant cyto-
plasm arranged in pseudorosettes
(B) Pleomorphic anaplastic cells with foci of
necrosis in a palisading pattern
(C) Regular round cells aligned smoothly with
spherical nuclei surrounded by clear cyto-
plasm and finely granular chromatin asso-
ciated with calcifications
(D) Stratified squamous epithelial cells embed-
ded in spongy reticular stroma with promi- Image courtesy of Armed Forces Institute of Pathology.
nent peripheral gliosis
(E) Whorls of meningothelial cells with oval-
shaped nuclei with indistinct cytoplasm (A) Follicular carcinoma
and psammoma bodies (B) Medullary carcinoma

Hematology-Oncology
(C) Multinodular goiter
8. A 17-year-old boy presents to the emergency (D) Papillary carcinoma
department with severe abdominal pain. Labo- (E) Thyroglossal duct cyst
ratory tests show a deficit in uroporphyrinogen
I synthetase and excess δ-aminolevulinate and 10. A 7-year-old African-American boy is brought
porphobilinogen in the urine. Which of the to see his pediatrician. His father says he has
following symptoms would most likely also be noticed that the boy has been complaining of
present in this patient? right knee pain for the past week. On physical
(A) Chest pain exam, multiple ecchymoses are noted on both
(B) Hypotension upper and lower extremities. His father claims
(C) Neuropsychiatric disturbances that the boy has always bruised easily, and he
(D) Polyphagia has recently learned how to ride a bicycle.
(E) Stiff neck Which of the following elements of the coagu-
lation cascade is most likely to be missing in
9. A 35-year-old woman visits her primary care this child?
physician after feeling a hard lump in her (A) Antithrombin III
neck. Her physician notes that she has a single, (B) Factor VII
hard, nontender nodule in the left lobe of her (C) Factor VIII
thyroid that moves when she swallows. There (D) Factor IX
is no cervical lymphadenopathy. The patient (E) Protein C
denies any changes in her recent health. The
 250 Section II: Organ Systems • Questions

11. A 16-year-old boy is brought to the emergency (A) Acute myeloid leukemia
department because of the acute onset of fever, (B) Chronic idiopathic myelofibrosis
chills, and a productive cough. X-ray of the (C) Chronic lymphocytic leukemia
chest shows an infiltrate restricted to the left (D) Chronic myelogenous leukemia
lower lobe. Samples taken of the sputum show (E) Hairy cell leukemia
α-hemolytic gram-positive cocci in pairs. The
patient says that he has had similar infections 13. During autopsy of a 65-year-old woman, the
over the past year. A peripheral blood smear is liver is examined, revealing multiple tumors of
done, and results show several sickle-shaped various sizes throughout both lobes. This pat-
RBCs. Which of the following explains why tern, along with the fact that most tumors found
this patient is susceptible to this particular type in the liver are metastases, leads the pathologist
of infection? to suspect that a primary tumor exists in an-
HIGH-YIELD SYSTEMS

other organ. Which of the following is the most

(A) Bone marrow infiltration resulting in neu-
likely location of the primary tumor?
tropenia and compromised immune func-
tion (A) Breast
(B) Large vessel occlusions in the cerebral vas- (B) Colon
culature resulting in neurologic events and (C) Kidney
aspiration pneumonia (D) Lung
(C) Microvascular infarcts resulting in pulmo- (E) Thyroid
nary failure
(D) Microvascular infarcts resulting in splenic 14. A child is brought to the pediatrician because
dysfunction her parents are concerned about lead poison-
(E) Vaso-occlusion in the renal medulla ing since their house is known to contain lead-
based paint. A complete blood cell count re-
12. A 55-year-old man presents to his physician veals anemia. Lead poisoning causes anemia
with easy bruising, splenomegaly, and fatigue. because it does which of the following?
A peripheral blood smear is shown in the im-
Hematology-Oncology

(A) Disrupts heme synthesis by causing de-

age. Which of the following is the most likely creased iron absorption from the gut
diagnosis? (B) Disrupts heme synthesis by increasing the
activity of aminolevulinate dehydratase
(C) Disrupts heme synthesis by inhibiting fer-
rochelatase
(D) Disrupts hemoglobin function by binding
to hemoglobin with high affinity, prevent-
ing oxygen binding
(E) Disrupts RBC DNA synthesis, causing
megaloblastic changes in RBCs

15. A 10-year-old boy with jaundice, splenomegaly,

and chronic normocytic, normochromic ane-
mia with an abnormal osmotic fragility test and
increased mean corpuscular hemoglobin con-
centration presents to the clinic for a consult.
Reproduced, with permission, from Lichtman MA, Beutler E,
He has had these symptoms for most of his life,
Kipps TJ, Seligsohn U, Kaushansky K, Prchal JF. Williams He- and his parents are requesting a definitive cure.
matology, 7th ed. New York: McGraw-Hill, 2006: Color Plate Which of the following confers a definitive
XVI-2. cure for this patient’s disease?
 Chapter 11: Hematology-Oncology • Questions 251

(A) Blood transfusions 18. Breast cancer is the leading cause of cancer in
(B) Bone marrow transplant women in the United States and the second
(C) Folate therapy leading cause of cancer deaths in women. Age,
(D) Iron therapy a family history of breast cancer, and mutations
(E) Splenectomy in the BRCA1 and BRCA2 genes are impor-
tant risk factors for the development of breast
16. A 60-year-old woman presents to her physician cancer. Which of the following drugs is used as
with findings of decreased proprioception in prophylaxis in women at high risk for develop-
her lower extremities and gait instability con- ing breast cancer?
sistent with subacute combined degeneration
(A) Abciximab and aspirin
secondary to cobalamin deficiency. However,
(B) Flutamide and leuprolide
blood tests show normal hematocrit and near-
(C) Tamoxifen and raloxifene

HIGH-YIELD SYSTEMS
normal mean cell volume. The physician or-
(D) Testosterone and prednisone
ders additional tests. Which of the following
(E) Vincristine and vinblastine
laboratory results would support a diagnosis of
cobalamin deficiency?
19. A 47-year-old woman from the Middle East
(A) Decreased level of lactate dehydrogenase presents to the clinic with fever, general mal-
(B) Elevated methylmalonic acid aise, and weight loss. Physical examination re-
(C) Elevated WBC count veals hepatomegaly and massive splenomegaly,
(D) Increased cobalamin levels along with edema. Laboratory tests show mod-
(E) Microcytosis erate anemia and a peripheral WBC count
(F) Negative anti-intrinsic factor antibody <4000/mm3. Macrophages containing amastig-
(G) Negative Schilling test otes are seen on histologic analysis. Which of
the following parasites does the woman most
17. A 73-year-old woman is brought to the emer- likely harbor?
gency department after passing out on the sub-
(A) Babesia species
way. The patient has a 3-month history of burn-

Hematology-Oncology
(B) Entamoeba histolytica
ing epigastric pain that is worse after eating,
(C) Giardia lamblia
along with dark stool. Physical examination
(D) Leishmania donovani
shows a blood pressure of 95/52 mm Hg, a
(E) Plasmodium ovale
pulse of 97/min, epigastric tenderness, and
guaiac-positive stool. Laboratory tests show a
20. A type of lymphoma is characterized by onset
hemoglobin level of 9.5 g/dL, a hematocrit of
in middle age and by neoplastic cells that re-
28%, and a mean corpuscular volume of 75
semble normal germinal center B lymphocytes.
µm3. Which of the following is the most likely
Additionally, it is the most common type of
diagnosis?
non-Hodgkin’s lymphoma in the United States.
(A) Anemia of chronic disease secondary to What is the characteristic chromosomal trans-
peptic ulcer disease location and protein that is produced by this
(B) Iron deficiency anemia secondary to blood translocation?
loss
(A) t(8:14), c-myc
(C) Iron deficiency anemia secondary to perni-
(B) t(9:22), bcr-abl
cious anemia
(C) t(11:22), EWS-FL-1
(D) Megaloblastic anemia secondary to blood
(D) t(14:18), bcl-2
loss
(E) t(15:17), PML-RAR-α
(E) Megaloblastic anemia secondary to perni-
cious anemia
 252 Section II: Organ Systems • Questions

21. A 52-year-old heart transplant patient on small intestine and then binds to receptors
chronic immunosuppressive therapy that in- on the wall of the ileum
cludes cyclosporine develops bacterial sinusitis. (D) Splits R-protein/vitamin B12 complexes in
The patient’s physician decides to start him on the duodenum
antibiotic therapy but is having difficulty (E) Splits vitamin B12 from its exogenous in-
choosing between amoxicillin and erythromy- gested protein-bound form
cin to treat the infection. Concurrent use of
erythromycin and cyclosporine would most 24. A 2-year-old boy is brought to a clinic because
likely lead to which of the following serum his mother noticed a large, unilateral, painless
drug levels? abdominal mass while she was bathing him.
While performing an ultrasound-guided bi-
(A) Decreased cyclosporine serum concentra-
opsy, the technician notes that the kidney caly-
tions
HIGH-YIELD SYSTEMS

ces are highly distorted by the mass. Which of

(B) First decreased, then increased serum cy-
the following is most likely the origin of this le-
closporine concentrations
sion?
(C) First increased, then decreased serum cy-
closporine concentrations (A) An autosomal dominant gene that also
(D) Increased cyclosporine serum concentra- leads to cystic masses in the liver
tions (B) Embryonic renal cells from the embryonic
(E) Unchanged cyclosporine serum concen- kidney
trations (C) Malignant transformation of renal tubular
cells
22. A 56-year-old man is diagnosed with transi- (D) Malignant transformation of uroepethilial
tional cell carcinoma and treated with excision cells
and chemotherapy. Which of the following risk (E) Primitive neural crest cells
factors most likely led to this patient’s cancer?
25. An 8-year-old boy is brought to the pediatrician
(A) Alcohol use
after his parents noticed very dark urine in the
Hematology-Oncology

(B) Exposure to aniline dyes

toilet earlier that morning. Initial laboratory
(C) Exposure to asbestos
studies show intravascular hemolysis; further
(D) Hypertension
testing shows that his RBCs are susceptible to
(E) Previous pyelonephritis
complement-mediated lysis. This patient most
likely has which of the following conditions?
23. A 70-year-old man with no prior surgical his-
tory presents with a tingling sensation of the (A) Autoimmune hemolytic anemia
extremities, weakness, and an enlarged tongue (B) Common bile duct stricture
that is sore and smooth. Preliminary laboratory (C) Hereditary spherocytosis
studies are negative for antinuclear antibodies (D) Paroxysmal nocturnal hemoglobinuria
and antimitochondrial antibodies, and periph- (E) Thrombotic thrombocytopenic purpura
eral blood smear reveals a macrocytic megalo-
blastic anemia. When the patient is given an 26. A 58-year-old man has a past medical history
oral dose of radiolabeled cyanocobalamin, significant for chronic renal insufficiency. He
none of that compound is detected in his was recently diagnosed with transitional cell
urine. What is the function of the protein that carcinoma of the bladder and will be started
is most likely deficient and/or affected in this on chemotherapy. Which of the following che-
disease process? motherapeutic agents should be avoided in this
patient?
(A) Binds free vitamin B12 in ileal cells for
transport through the bloodstream (A) Bleomycin
(B) Binds free vitamin B12 in the stomach (B) Cisplatin
(C) Complex with free vitamin B12 in the (C) Cytarabine
 Chapter 11: Hematology-Oncology • Questions 253

(D) Doxorubicin ing of fatigue and general exhaustion. Physical

(E) Tamoxifen examination reveals that her sclerae are icteric,
her palate is abnormally pigmented, and her
27. A 3-year-old girl was in her usual state of good skin has a yellow hue. Ultrasound shows ra-
health 1 month ago when she developed an diopaque gallstones. A Coombs’ test is nega-
acute viral upper respiratory infection. She tive. A peripheral blood smear shows small
now presents to the emergency department RBCs, several of which have no central pallor.
with nonblanching purple skin lesions. The Which of the following is the most likely cause
rest of her physical examination is unremark- of this patient’s condition?
able. The complete blood count demonstrates
(A) A mutation in the gene encoding ankyrin
a low platelet count, while the peripheral
(B) A mutation in the glucose 6-phosphate de-
blood smear is notable only for large platelets.
hydrogenase gene

HIGH-YIELD SYSTEMS
Which of the following laboratory findings
(C) Circulating antibodies targeted against
would most likely be present in this patient?
erythrocytes
(A) Antiplatelet antibodies (D) Iron deficiency anemia
(B) Decreased megakaryocytes on bone mar- (E) RBC hemolysis because of a mechanical
row biopsy heart valve
(C) IgA deficiency
(D) Increased fibrin split products 30. A research group is studying sickle cell disease
(E) Vitamin K deficiency in a small community of 6000 people in cen-
tral Africa and performs a genetic analysis on
28. A 57-year-old woman is diagnosed with small- every community member. At the beginning of
cell lung carcinoma. She has gained weight, the year, it is determined that 10% are homozy-
and she says that her face has recently “bal- gous for hemoglobin S and therefore have
looned.” Which of the following symptoms is sickle cell disease. Thirty percent of the com-
this patient likely to be experiencing in addi- munity is heterozygous for the mutant allele.
tion to the ones mentioned? Over the course of the year, 100 infants are

Hematology-Oncology
(A) Cold intolerance and hair loss born, six of whom are diagnosed with sickle
(B) Galactorrhea and amenorrhea cell disease. Of the 80 people who die during
(C) Insatiable thirst and polyuria the year, three had sickle cell disease. Which
(D) Poor wound healing and hirsutism of the following describes the current preva-
(E) Restlessness and tremor lence of homozygous sickle cell disease in this
population?
29. A 45-year-old woman arrives in the emergency (A) 3/6020
department complaining of intense pain in her (B) 6/100
upper abdomen for the past 4 hours. She had a (C) 6/6020
similar episode in the past, but it went away (D) 603/6020
within an hour. Her history is significant for a (E) 606/6100
recent flu-like infection and a prolonged feel-
 254 Section II: Organ Systems • Questions

31. A 65-year-old man comes to a local clinic com- (D) Gatifloxacin

plaining of back pain. After ruling out the most (E) Trimethoprim
common causes of back pain, the treating phy-
sician becomes suspicious of a malignant pro- 33. A 60-year-old woman presents to the physician
cess. He sends the patient for a bone marrow because of red-brown streaks of blood in her
biopsy, with the results shown in the image. stool. She reports no pain with bowel move-
Which of the following is the most likely diag- ments. Initial laboratory tests show:
nosis?
Hematocrit: 33%
Hemoglobin: 11 g/dL
Mean corpuscular volume: 73 µm³
WBC count: 8000/mm³
Platelet count: 200,000/mm³
HIGH-YIELD SYSTEMS

Which of the following is the most important

next step in management?
(A) Abdominal plain films
(B) Colonoscopy
(C) CT scan of the abdomen
(D) Esophagoduodenoscopy
(E) Pelvic ultrasound

Reproduced, with permission, from Lichtman MA, Beutler E, 34. A patient with relapsing Hodgkin’s disease pres-
Kipps TJ, Seligsohn U, Kaushansky K, Prchal JF. Williams ents with weight gain, foot ulcers, vision prob-
Hematology, 7th ed. New York: McGraw-Hill, 2006: Color lems, elevated blood sugar, oral candidiasis,
Plate XXI-2. and new onset of wildly swinging mood
changes. What is the most likely etiology of
this patient’s psychiatric symptoms?
Hematology-Oncology

(A) Bone marrow suppression

(B) Lymphoma (A) Adverse effects of bleomycin
(C) Metastatic prostate cancer (B) Adverse effects of prednisone
(D) Multiple myeloma (C) Adverse effects of vincristine
(E) Normal bone marrow (D) Normal psychiatric response to having
cancer
32. A 23-year-old woman presents to the emer- (E) Progression of disease
gency department following a motor vehicle
crash. Her injuries are minor, and she states 35. A 41-year-old pregnant woman sees her obste-
that she feels a bit dizzy and tired but has felt trician because of new-onset vaginal bleeding.
this way recently anyway. The physician no- Although she is only 4 months pregnant, her
tices that her laboratory results are consistent doctor notes that her uterus is the size usually
with megaloblastic anemia. Upon further ques- seen at 6 months of gestation. Maternal blood
tioning, she reports that she has been taking works shows a β-human chorionic gonadotro-
antibiotics off and on over the last several pin (β-hCG) level >5 times the upper limit of
months for frequent urinary tract infections. normal. If left untreated, what is a possible
Which of the following medications is this pa- consequence of the patient’s condition?
tient most likely taking? (A) Choriocarcinoma
(A) Chloramphenicol (B) Coma
(B) Clindamycin (C) Fetal neural tube defects
(C) Erythromycin (D) Ovarian cancer
(E) Uterine bleeding and exsanguination
 Chapter 11: Hematology-Oncology • Questions 255

36. Hydrops fetalis occurs in the setting of a cer- (C) Plasma cell
tain type of thalassemia. What is the underly- (D) Reed-Sternberg cell
ing mechanism leading to this event? (E) T lymphocyte
(A) Excess α-globin chains binding tighter to
39. A 29-year-old man presents to his primary care
oxygen
physician with a painless testicular mass. Labo-
(B) Excess α-globin chains binding weaker to
ratory studies show an elevated serum human
oxygen
chorionic gonadotropin level. Which of the
(C) Excess β-globin chains binding tighter to
following is the most likely site of nodal meta-
oxygen
stasis in this tumor?
(D) Excess β-globin chains binding weaker to
oxygen (A) Deep inguinal lymph nodes
(E) Excess gamma-globin chains binding (B) External iliac lymph nodes

HIGH-YIELD SYSTEMS
tighter to oxygen (C) Gluteal lymph nodes
(F) Excess gamma-globin chains binding (D) Para-aortic lymph nodes
weaker to oxygen (E) Superficial inguinal lymph nodes

37. A 56-year-old man who is a health care worker 40. A 28-year-old woman comes to the physician
presents to his physician with vague abdominal concerned about an excessive amount of bleed-
discomfort. A physical examination reveals a ten- ing from her gums when she brushes her teeth.
der liver, palpable to 6 cm below the costal mar- Her laboratory results show an increased partial
gin and scleral icterus. His laboratory studies are thromboplastin time and an increased bleed-
significant for an aspartate aminotransferase ac- ing time, but are otherwise unremarkable.
tivity of 200 U/L and an alanine aminotrans- Which of the following treatments will most
ferase activity of 450 U/L. A CT scan of the ab- likely alleviate this patient’s symptoms?
domen shows a dominant solid nodule in the (A) Cryoprecipitate
liver. The marker most likely to be elevated in (B) Factor VIII concentrate
this patient is also a good indicator of which of

Hematology-Oncology
(C) Fresh frozen plasma
the following malignancies? (D) Low-molecular-weight heparin
(A) Choriocarcinoma (E) Protamine sulfate
(B) Colorectal carcinoma
(C) Melanoma 41. A 29-year-old woman, who is 32 weeks’ preg-
(D) Neuroblastoma nant and has been in the hospital for 3 days be-
(E) Prostatic carcinoma cause of pyelonephritis, starts oozing blood
(F) Yolk sac carcinoma from her intravenous lines and bleeding from
her gums. Petechiae are also noted in her skin.
38. A 57-year-old man presents to his physician Laboratory tests show a platelet count of
with a 4-month history of worsening fatigue 98,000/mm³, hematocrit of 38%, WBC count
and generalized weakness. Further questioning of 8000/mm³, and a prolonged prothrombin
reveals that his clothes fit him more loosely time. What other laboratory anomaly would
now than they had in the past. Physical exami- also be expected?
nation reveals generalized lymphadenopathy (A) Elevated D-dimer levels
and hepatosplenomegaly. Lymph node biopsy (B) Elevated factor VII levels
specimens are sent to the pathologist with the (C) Elevated fibrinogen levels
presumptive diagnosis of lymphoma. Which of (D) Elevated protein C levels
the following types of neoplastic cell is most (E) Elevated protein S levels
common in non-Hodgkin’s lymphoma?
(A) B lymphocyte
(B) Myeloblast
 256 Section II: Organ Systems • Questions

42. A 62-year-old woman presents to the clinic (D) Selegiline

complaining of frequent bleeding while brush- (E) Ticlopidine
ing her teeth and easy bruising. She reports
she recently had pneumonia and was treated 45. A 49-year-old man presents to the emergency
with a broad-spectrum antibiotic. Laboratory department complaining that “my skin has
tests show: turned yellow.” Physical examination reveals
the man is significantly jaundiced. He has no
Prothrombin time: 18 seconds abdominal pain and has a negative Murphy’s
Partial thromboplastin time: 37 seconds sign. The physician is concerned that he can
Platelet count: 231,000/mm³ feel the patient’s gallbladder and orders a CT
Hematocrit: 37% scan. What is the most likely cause of this pa-
WBC count: 4800/mm³ tient’s jaundice?
HIGH-YIELD SYSTEMS

The cofactor that is deficient in this patient is (A) Acute hepatitis

needed for the carboxylation of glutamate resi- (B) Choledocholithiasis
dues of which of the following? (C) Cholelithiasis
(A) Factors II, VII, VIII, and X (D) Hemolytic anemia
(B) Factors VII, VIII, IX, and XII (E) Pancreatic cancer
(C) Proteins C and S and factors IX, X, XI, and
46. A 34-year-old man comes to the emergency de-
XII
partment complaining of the sudden onset of
(D) Proteins C and S and factors XII, IX, and X
vomiting and epigastric abdominal pain radiat-
(E) Proteins C and S, prothrombin, and fac-
ing to the back. On physical examination, the
tors VII, IX, and X
patient is afebrile and has abdominal tender-
43. A 20-year-old African-American man develops ness; decreased bowel sounds are noted, as is
anemia after being treated for a urinary tract in- diffuse bruising that he describes as having ap-
fection. A peripheral blood smear shows RBC peared suddenly. He also reports continuous
epistaxis. Laboratory tests show a slightly ele-
Hematology-Oncology

lysis and precipitates of hemoglobin within the

RBCs. Which of the following drug classes vated WBC count, thrombocytopenia, in-
most likely caused his hemolytic anemia? creased amylase and lipase activity, increased
prothrombin time (PT) and partial thrombo-
(A) Aminoglycosides plastin time (PTT), and the presence of fibrin
(B) Fluoroquinolones split products. An abdominal ultrasound per-
(C) Macrolides formed at the bedside shows a dilated common
(D) Sulfonamides bile duct. Which of the following is the most
(E) Tetracyclines likely etiology of this patient’s abnormal coagu-
lation profile?
44. Several drugs are used to prevent myocardial
infarction in patients with acute coronary syn- (A) Acute hepatitis
drome. One class of drugs binds to the glyco- (B) Acute pancreatitis
protein receptor IIb/IIIa on activated platelets, (C) Appendicitis
thereby interfering with platelet aggregation. (D) Perforated gastric ulcer
This prevents renewed formation of clots that (E) von Willebrand’s disease
could block the lumen of the cardiac vessels.
Which of the following is an example of this 47. A 70-year-old man comes to his physician for a
class of drug? routine physical examination. Although he is
asymptomatic, a blood test shows an abnormal
(A) Abciximab level of immunoglobulin. After further testing,
(B) Clopidogrel he is diagnosed with monoclonal gammopathy
(C) Leuprolide of undetermined significance. Which of the
 Chapter 11: Hematology-Oncology • Questions 257

following is the current treatment for monoclo- cerebellar hemisphere. He reports a 3-month
nal gammopathy of undetermined signifi- history of dizziness and a 4-year history of pro-
cance? gressive hearing loss. Grossly, the tumor speci-
men appears as a single irregular fragment of
(A) Alendronate
tan-pink soft tissue that measures slightly less
(B) Anticoagulation
than 2 cm. A microscopic pathology report in-
(C) High-dose steroids
dicates that the specimen consists of compact
(D) No treatment
areas of spindle cells with pink cytoplasm that
(E) Vinca alkaloids
form whirls and palisades. Which of the follow-
48. An 8-year-old boy has a chronic history of se- ing types of tumors would most likely result in
vere hemolytic anemia, hepatosplenomegaly, these findings?
and maxillary overgrowth. He has received (A) Medulloblastoma

HIGH-YIELD SYSTEMS
blood transfusions since early infancy but has (B) Meningioma
not received a transfusion in over 4 months. A (C) Neurofibroma
hemoglobin electrophoresis shows marked ele- (D) Oligodendroglioma
vation of HbF, increased HbA2, and absence of (E) Schwannoma
HbA1. Which of the following diagnoses is
most consistent with this patient’s electropho- 50. A 22-year-old man is diagnosed with medullary
resis? thyroid carcinoma, and his blood tests are sig-
nificant for hypercalcemia and increased
(A) α-Thalassemia minor
plasma catecholamine levels. He reports that
(B) β-Thalassemia major
his mother also had medullary thyroid carci-
(C) β-Thalassemia minor
noma in her twenties and that she recently had
(D) Glucose 6-phosphate dehydrogenase defi-
surgery to remove her parathyroid glands.
ciency
These findings suggest a possible mutation in
(E) HbH disease
which of the following genes?
(F) RBCs containing hemoglobin Barts

Hematology-Oncology
(A) erb-B2
49. A 69-year-old man has a tumor removed from (B) MEN I
the cerebellopontine angle because a CT scan (C) ras
shows a 2-cm sharply circumscribed mass adja- (D) Rb
cent to the right pons extending into the right (E) ret
 258 Section II: Organ Systems • Answers

AN S W E R S

1. The correct answer is A. In the vertical growth patient because she is a smoker, which is inde-
phase of melanoma, neoplastic cells grow pendently a significant risk factor for transi-
deeply into the dermis. Considering that inva- tional cell carcinoma.
sion of lymphatics or blood vessels (deep struc-
Answer A is incorrect. Leucovorin can be used
tures) is necessary for distant metastasis of any
to reverse the myelosuppression that may oc-
cancer, it can easily be understood why the
cur with the use of methotrexate. Although
depth of invasion is so important in highly la-
methotrexate is likely one of the drugs that the
bile melanoma. The depth of invasion into the
patient will receive, there is no need to reverse
dermis is an established prognostic factor for
its action until there is clear evidence of prob-
the patient, since most melanoma patients die
HIGH-YIELD SYSTEMS

lematic immunosuppression. Conversely, ad-

due to complications of metastatic melanoma
ministering mesna from the start is appropriate
involvement of other organs (lung or brain).
and makes sense, as it is protective of the blad-
Patients with a tumor depth <1.7 mm have a
der.
more favorable prognosis. Remember the AB-
CDE’s of melanoma: Asymmetry, Borders (ir- Answer C is incorrect. Thymidine can be used
regular), Color variation, Diameter >6 mm, to reverse the myelosuppression that may oc-
and Elevation/Evolution. cur with the use of 5-FU. Although 5-FU is
likely one of the drugs that the patient will re-
Answer B is incorrect. Location of the lesion
ceive, there is no need to reverse its action un-
on an extremity, rather than the central body,
til there is clear evidence of problematic im-
indicates a more favorable prognosis.
munosuppression. Conversely, administering
Answer C is incorrect. Lateral spread within mesna from the start is appropriate and makes
the epidermis is not as important as depth in sense, as it is protective of the bladder.
determining metastatic potential.
Answer D is incorrect. Coadministration of vi-
Hematology-Oncology

Answer D is incorrect. Previous lesions do not tamin B12 would not be helpful for this patient.
have an impact on the likelihood of this nevus However, administration of vitamin B12 is nec-
to metastasize. essary for a vitamin B12 deficiency caused by
malabsorption, pernicious anemia, or a dis-
Answer E is incorrect. Size of neoplastic cells
eased terminal ileum.
is not a factor in determining prognosis.
Answer E is incorrect. Coadministration of vi-
2. The correct answer is B. The patient has inva- tamin B6 would not be helpful for this patient.
sive ductal carcinoma, which must be resected However, coadministration of vitamin B6 can
and then treated with chemotherapy. There prevent neurotoxicity in a patient taking isoni-
are many chemotherapy combinations used to azid for tuberculosis.
treat breast cancer, two of the most common
being (1) cyclophosphamide, methotrexate, 3. The correct answer is B. The question stem
and 5-fluorouracil (5-FU) and (2) cyclophos- describes a case of a pancreatic gastrinoma
phamide, doxorubicin, and 5-FU. Almost all of leading to Zollinger-Ellison syndrome. This
the regimens use the drug cyclophosphamide, syndrome results from hypergastrinemia. Gas-
an alkylating agent, which is commonly also trin is normally produced by the G cells of the
used to treat non-Hodgkin’s lymphoma and antrum and duodenum. For example, ectopic
ovarian cancer. The most important toxicity of and unregulated production of gastrin by ma-
this drug is hemorrhagic cystitis, which can lignant pancreatic islet cells stimulates excess
lead to transitional cell carcinoma. Hemor- secretion of acid by parietal cells of the stom-
rhagic cystitis is prevented by coadministration ach. Gastrin also promotes hypertrophy of the
of mesna. This is especially important in this stomach. Increased stomach acid leads to
 Chapter 11: Hematology-Oncology • Answers 259

symptoms of gastroesophageal reflux disease Answer D is incorrect. A tumor in the right

and to ulceration and gastric bleeding, as evi- lower lobe would not manifest in these symp-
denced by the patient’s pallor and melena. El- toms.
evated serum gastrin would be diagnostic of
Answer E is incorrect. A tumor located in the
gastrinoma.
right middle lobe could not result in these
Answer A is incorrect. Cholecystokinin (CCK) symptoms because it would not be located near
is produced by the I cells of the duodenum and the cervical sympathetic plexus.
jejunum. CCK stimulates gallbladder contrac-
tion and pancreatic enzyme secretion. CCK 5. The correct answer is D. Late menopause
inhibits gastric acid secretion. (>50 years old) is associated with an increased
risk of breast cancer. Other risk factors include
Answer C is incorrect. Intrinsic factor is also
being female, older age, early first menarche

HIGH-YIELD SYSTEMS
released by the parietal cells of the stomach; its
(<12 years old), delayed first pregnancy (>30
function is to bind vitamin B12 to enable its ab-
years old), and family history of a first-degree
sorption. High intrinsic factor levels would be
relative with breast cancer at a young age.
unlikely to produce the symptoms of elevated
gastrin secretion in this case. Answer A is incorrect. Breast cancer risk is not
increased by caffeine intake.
Answer D is incorrect. Secretin is produced
normally by the S cells of the duodenum and Answer B is incorrect. Breast cancer risk is not
promotes pancreatic bicarbonate secretion increased by nonhyperplastic cysts. Had the
while inhibiting gastric acid secretion. cyst drained bloody fluid, it would have been
more worrisome for a malignant process.
Answer E is incorrect. Somatostatin is pro-
duced by the pancreatic islets and gastrointesti- Answer C is incorrect. Breast cancer risk is not
nal mucosa. Unlike gastrin, somatostatin inhib- increased by fibroadenoma.
its gastric acid and pepsinogen secretion in Answer E is incorrect. Breast cancer risk is not
addition to counteracting the actions of CCK increased by use of St. John’s wort.

Hematology-Oncology
and secretin. Somatostatin also inhibits the re-
lease of insulin and glucagons. 6. The correct answer is D. LMWHs act predomi-
nantly on Xa, whereas unfractionated heparin
4. The correct answer is B. The question stem targets both Xa and IIa. A major advantage of
describes a case of Pancoast’s tumor, in which using LMWHs over unfractionated heparin is
a lung neoplasm of any type located in the that there is no need for monitoring of APTT.
apex impinges on the cervical sympathetic
plexus. This impingement results in Horner’s Answer A is incorrect. Heparin targets both
syndrome of ptosis, miosis, and anhidrosis, as antithrombin III and Xa.
described in this patient. The symptoms are Answer B is incorrect. Factor IIa, or thrombin,
ipsilateral to the damaged plexus, so the tumor is the enzyme that catalyzes the final step of the
must be located in the apex of the right upper clotting cascade, the formation of fibrin. Throm-
lobe. bin is a vitamin K-dependent factor that is not
Answer A is incorrect. The symptoms are ipsi- influenced by low-molecular-weight heparin.
lateral to the injured plexus, so a tumor in the Answer C is incorrect. Factor VII is part of the
left apex would have presented with Horner’s extrinsic pathway and is one of the vitamin
syndrome on the left side, not the right. K-dependent factors.
Answer C is incorrect. Squamous cell carci- Answer E is incorrect. Warfarin blocks the ac-
noma of the lung usually appears at the hilum, tivation of vitamin K-dependent factors II, VII,
but such a tumor mass would not cause IX, and X.
Horner’s syndrome.
 260 Section II: Organ Systems • Answers

7. The correct answer is A. The diagnosis is lations. Clinically these tumors often present
medulloblastoma, which occurs predominantly with seizures or a neurological deficit that
in children and is found exclusively in the cer- gradually worsens over time. Focal symptoms
ebellum, accounting for 20% of brain tumors vary by location. These tumors are slow grow-
in children. On microscopic examination the ing and expand locally rather than by infiltra-
tumor is very cellular with sheets of anaplastic tion.
cells. Each tumor cell is small with little cyto-
plasm and crescent-shaped, deeply staining nu- 8. The correct answer is C. This patient suffers
clei (due to high mitotic activity) arranged in from acute intermittent porphyria (AIP), an au-
pseudorosettes. These tumors are frequently ra- tosomal dominant disorder caused by a lack of
diosensitive. uroporphyrinogen I synthetase. The buildup of
toxic levels of δ-aminolevulinate (ALA) and
Answer B is incorrect. This micropathologic
HIGH-YIELD SYSTEMS

porphobilinogen lead to the associated symp-

description is characteristic of glioblastoma
toms of abdominal pain (more than 90% of
multiforme, which is the most common pri-
cases), neuropathy, high sympathetic tone, and
mary brain tumor of adults and occurs in the
neuropsychiatric disturbances, including anxi-
cerebral hemispheres. It carries a particularly
ety, depression, seizures, and paranoia. AIP al-
grave prognosis, with a survival rate at 5 years
most never presents before puberty, and it can
of almost zero. Most patients live less than 1
be hard to diagnose because of its acute nature.
year after diagnosis. This is seldom a tumor of
Untreated, it can lead to paralysis and death.
childhood and typically presents with severe
headache and symptoms consistent with in- Answer A is incorrect. The differential diagno-
creased intracranial pressure. sis for chest pain is long and includes cardiac,
pulmonary, gastrointestinal, and musculoskele-
Answer C is incorrect. This description is con-
tal etiologies. However, attacks of acute inter-
sistent with a diagnosis of oligodendroglioma,
mittent porphyria are not associated with chest
which is a relatively rare, slow-growing, benign
pain.
tumor that is most often found in the frontal
Hematology-Oncology

lobes. The clinical features are nonspecific and Answer B is incorrect. Due to the high sympa-
vary based on the location and extent of dis- thetic tone caused by the pain of the crisis, hy-
ease. General symptoms include headaches, pertension may be associated with acute inter-
seizures, and changes in cognition, while focal mittent porphyria, but not hypotension.
lesions can present with localized weakness, Answer D is incorrect. Polyphagia, or greatly
sensory loss, or aphasia. The micropathologic increased hunger, is one of the cardinal symp-
appearance is classically described as consist- toms associated with diabetes mellitus, not
ing of “fried egg” cells that have round nuclei acute intermittent porphyria.
with clear cytoplasm and are often calcified.
Answer E is incorrect. A stiff neck may be as-
Answer D is incorrect. This histopathology de- sociated with meningeal irritation and can be
scribes a craniopharyngioma, the most com- found in meningitis or with musculoskeletal
mon supratentorial tumor in children. It is em- problems, but it is not found in acute intermit-
bryologically derived from remnants of Rath- tent porphyria.
ke’s pouch. The clinical presentation typically
involves severe headaches, visual changes, and 9. The correct answer is D. This patient has pap-
pituitary dysfunction (growth failure in chil- illary carcinoma, the most common form of
dren). It has a bimodal distribution of affected thyroid cancer. Patients are frequently in their
ages, with a second peak in the fifth decade of 20s–40s and many have a history of exposure
life. to ionizing radiation. Most papillary carcino-
Answer E is incorrect. This is a description of mas are cold masses on scintiscan. On histo-
a meningioma, a nonmalignant primary intrac- logic exam, papillary carcinomas show branch-
ranial neoplasm arising from arachnoid granu- ing papillae with a fibrovascular stalk. These
 Chapter 11: Hematology-Oncology • Answers 261

papillae are lined by epithelial cells with Answer E is incorrect. A thyroglossal cyst is a
empty-looking, ground-glass nuclei often called congenital abnormality caused by the persis-
“Orphan Annie eye” nuclei. Also characteristic tence of the diverticulum formed during the
of papillary carcinoma is the concentrically migration of the thyroid gland from the fora-
calcified psammoma bodies shown in the im- men cecum to the tracheal cartilage. These le-
age above. Psammoma bodies are almost never sions are found in the midline and do not lo-
seen in follicular or medullary carcinomas. calize to one lobe.
The prognosis of papillary carcinoma is excel-
lent. The overall 10-year survival rate is about 10. The correct answer is C. This patient has he-
98%, especially in patients without lymph node mophilia A, an X-linked disorder caused by fac-
metastases. tor VIII deficiency. Bleeding frequently occurs
in joints and the retroperitoneal space. It is the
Answer A is incorrect. Follicular carcinoma is

HIGH-YIELD SYSTEMS
most common inherited coagulopathy, as
the second most common form of thyroid can-
1:10,000 males has a deficiency or dysfunction
cer and generally presents in women in their
of factor VIII.
40s and 50s. Follicular carcinomas may present
as well-circumscribed nodules or infiltrative le- Answer A is incorrect. Mutations leading to
sions. Like papillary carcinomas, they show up antithrombin III deficiency result in a rare
as cold lesions on scintigrams; however, their cause of inherited hypercoagulability. The his-
histology is different. Microscopically, follicu- tory of multiple ecchymoses on both upper and
lar carcinomas present as uniform cells sur- lower extremities and easy bruising argues
rounding colloid-filled follicles with Hurthle against a hypercoaguable disorder.
cells (cells with abundant granular eosino- Answer B is incorrect. Factors II, VII, IX, and
philic cytoplasm). There are no psammoma X are vitamin K-dependent clotting factors.
bodies seen in follicular carcinoma. The prog- None of them is deficient in patients with he-
nosis is excellent, with 10-year survival rates mophilia A, and deficiencies of these factors
>92%. are exceedingly rare.

Hematology-Oncology
Answer B is incorrect. Medullary carcinoma is Answer D is incorrect. Factor IX deficiency is
a neoplasm of the parafollicular or C cells of the cause of hemophilia B, another X-linked
the thyroid. These cells secrete calcitonin, disorder. The incidence of hemophilia B (also
which causes increased serum calcitonin lev- called Christmas disease) is 1:100,000 in
els. Medullary carcinoma can be sporadic, in males.
which case it tends to be unilateral. Medullary
carcinoma may also be associated with multi- Answer E is incorrect. Protein C or protein S
ple endocrine neoplasia types IIA and IIB, in deficiency is another uncommon inherited
which case it tends to affect both the right and cause of hypercoagulability.
left lobes of the thyroid. On histologic exam,
sheets of spindle-shaped cells are seen in amy- 11. The correct answer is D. This patient has
loid stroma. sickle cell disease, as evidenced by the erythro-
cyte sickling in the peripheral blood smear. Pa-
Answer C is incorrect. Multinodular goiters tients with this disease are prone to microvas-
are asymmetrically enlarged thyroid glands that cular infarcts in several vascular beds in the
may be toxic or nontoxic, meaning that they body. Over time, multiple micro-infarcts in the
may or may not overproduce thyroid hormone. spleen result in functional asplenia, making
Multinodular goiter may cause dysphagia due patients particularly susceptible to infections
to compression of the esophagus. It may also with encapsulated organisms. This patient
cause compression of the large vessels in the presents with pneumonia caused by Streptococ-
neck. The presentation of multinodular goiter cus pneumoniae. This is an encapsulated bac-
does not correlate with the clinical picture pre- terium, which can be confirmed by a positive
sented in this case. quellung reaction.
 262 Section II: Organ Systems • Answers

Answer A is incorrect. Sickle cell patients are and include weakness and easy fatigue, infec-
at risk of having an aplastic crisis, often associ- tions, and hemorrhagic findings such as gingi-
ated with parvovirus B19 infection, during val bleeding, ecchymoses, epistaxis, or menor-
which time bone marrow activity is suppressed. rhagia. The prognosis for patients with AML is
Some patients may subsequently develop bone poor; many patients die within a few years of
marrow necrosis. Although these patients are at diagnosis.
risk of developing a host of infections, this par-
Answer B is incorrect. Chronic idiopathic my-
ticular complication is not specifically associ-
elofibrosis also can present with splenomegaly
ated with susceptibility to infections with en-
and fatigue; however, peripheral blood smear
capsulated organisms.
would show teardrop-shaped RBCs, nucleated
Answer B is incorrect. Strokes in sickle cell pa- red cell precursors, and granulocytic precursor
tients due to large vessel occlusion can be a se- cells without the presence of Auer rods.
HIGH-YIELD SYSTEMS

rious cause of morbidity. Aspiration pneumonia

Answer C is incorrect. Chronic lymphocytic
could be a complication of these neurologic
leukemia is more common in patients >60 years
events, but this does not explain increased sus-
old. The disease has an insidious onset, with pa-
ceptibility to infection with encapsulated bacte-
tients presenting early on with fatigue and
ria.
weight loss and later with lymphadenopathy and
Answer C is incorrect. Although micro- hepatosplenomegaly. The peripheral blood
infarcts in the pulmonary vasculature are a smear reflects a marked lymphocytosis, with a
chronic complication of sickle cell disease and typically normocytic, normochromic anemia. As
constitute a leading cause of death for these pa- the disease progresses, the platelet count may
tients, they are not responsible for the patient’s drop. Auer rods are not a feature of lymphocytic
susceptibility to infections with encapsulated lymphomas or leukemias.
organisms.
Answer D is incorrect. Characteristics of
Answer E is incorrect. The renal medulla is chronic myelogenous leukemia include lymph-
particularly prone to vaso-occlusive complica- adenopathy, splenomegaly, marked leukocyto-
Hematology-Oncology

tions in sickle cell patients; renal failure and sis, mature myeloid precursor cells, and mini-
uremia could be possible complications. This mal blasts and promyelocytes. Auer rods are
can result in isothenuria, in which the kidney not typically seen on peripheral blood smear.
is not fully able to concentrate the urine.
Answer E is incorrect. Although hairy cell leu-
kemia affects middle-aged men who tend to
12. The correct answer is A. The cytoplasmic in-
present with massive splenomegaly and fatigue,
clusions seen in the myelocytic precursors in
Auer rods are not present in leukemic cells of
the image are fused lysosomal granules known
this disease. Hairy cell leukemia derives its
as Auer rods. Release of these fused granules
name from the hairlike filamentous projections
may cause acute disseminated intravascular co-
characteristic of affected leukemic cells.
agulation (DIC) and fatal hemorrhage. Auer
rods define a cell as being of myelocytic lin-
13. The correct answer is B. The liver and lung
eage as opposed to lymphocytic and are seen
are the most common sites of metastasis (after
in acute myeloid leukemia (AML), especially
lymph nodes) due to the high blood flow
in the subgroup of acute promyelocytic leuke-
through these organs. Therefore, primary tu-
mia, also known as M3 in the French Ameri-
mors in any of the locations listed may metas-
can British system (FAB). AML is the most
tasize to the liver. However, given that the
common form of leukemia in adults and con-
blood vessels from the gastrointestinal tract
stitutes approximately one-fifth of acute leuke-
drain into the hepatic circulation, the most
mia cases in children. Symptoms of AML are
likely primary tumor that metastasizes to the
related to complications of pancytopenia (i.e.,
liver would be from a gastrointestinal source
anemia, neutropenia, and thrombocytopenia)
such as the colon.
 Chapter 11: Hematology-Oncology • Answers 263

Answer A is incorrect. Breast tumors may also an abnormal osmotic fragility test and increased
metastasize to the liver, but with less frequency mean corpuscular hemoglobin concentration
than gastrointestinal cancers. Breast metastases (MCHC). These are all characteristics of hered-
are more often found in brain and bone. itary spherocytosis, an intrinsic, extravascular
hemolysis due to spectrin or ankyrin defect.
Answer C is incorrect. Tumors of the kidney,
RBCs are small and round with no central pal-
such as renal cell carcinoma, metastasize to
lor. The osmotic fragility test detects hemolysis
the brain and bone, and less so to the liver.
by measuring the fraction of hemoglobin re-
Answer D is incorrect. Lung tumors often me- leased from RBCs at progressively more dilute
tastasize to brain and bone, and less often to salt concentrations. It detects hemolysis in
the liver. spherocytes at salt concentrations that do not af-
Answer E is incorrect. Thyroid cancer often fect normal RBCs. The MCHC measures the

HIGH-YIELD SYSTEMS
leads to bone metastases. concentration of hemoglobin in a given volume
of packed RBCs and is elevated only in heredi-
14. The correct answer is C. Lead inhibits tary spherocytosis. The definitive treatment of
δ-aminolevulinate (ALA) dehydratase and ferro- hereditary spherocytosis is splenectomy because
chelatase, preventing both porphobilinogen for- the spleen is responsible for the breakdown of
mation and the incorporation of iron into proto- the defective RBCs in this disease. Once elimi-
porphyrin IX, the final step in heme synthesis. nated, the spleen no longer hemolyzes the
Inhibition of both of these steps results in inef- RBCs, and thus, the anemia improves.
fective heme synthesis and subsequent micro- Answer A is incorrect. Blood transfusions are
cytic (hemoglobin-poor) anemia. indicated in hereditary spherocytosis for severe
Answer A is incorrect. Lead poisoning does anemia, but are only temporary and not defini-
not affect iron absorption from the gut. tive treatment.

Answer B is incorrect. Lead inhibits ALA de- Answer B is incorrect. Bone marrow trans-
hydratase, preventing porphyrin synthesis be- plant is not indicated for hereditary spherocyto-

Hematology-Oncology
yond the formation of ALA; it does not increase sis. Splenectomy is curative and poses signifi-
its actions. This causes ALA to accumulate in cantly less risks than bone marrow transplant.
the urine. Answer C is incorrect. Folate therapy is indi-
Answer D is incorrect. Lead does not have a cated for hereditary spherocytosis, but is not a
high affinity for hemoglobin. This type of pa- definitive treatment. It is only a supportive
thology is seen in carbon monoxide (CO) poi- treatment that does not eliminate the problem.
soning. CO binds to hemoglobin with much Answer D is incorrect. Iron therapy is not indi-
higher affinity than oxygen, resulting in de- cated in hereditary spherocytosis.
creased oxygen-carrying capacity.
Answer E is incorrect. Lead does not interrupt 16. The correct answer is B. In a minority of pa-
RBC DNA synthesis. Folate and/or vitamin B12 tients with cobalamin (vitamin B12) deficiency,
deficiencies disrupt DNA synthesis, specifically hematocrit and mean corpuscular volume can
thiamine synthesis, resulting in megaloblastic be normal. In these cases, laboratory testing for
changes in RBCs. elevated homocysteine and methylmalonic
acid can be used to make the diagnosis.
15. The correct answer is E. The patient is exhib- Answer A is incorrect. Lactate dehydrogenase
iting symptoms of a hemolytic anemia because would be increased in vitamin B12 deficiency
he is anemic and has jaundice, which results due to failed hematopoiesis.
from the breakdown of RBCs leading to the
formation of excess bilirubin. Moreover, his Answer C is incorrect. Neutropenia with hy-
anemia is normochromic and normocytic with persegmented neutrophils is typically seen in
vitamin B12 deficiency.
 264 Section II: Organ Systems • Answers

Answer D is incorrect. Decreased cobalamin Answer D is incorrect. The low mean corpus-
levels would support a diagnosis of vitamin B12 cular volume is not consistent with megalo-
deficiency. blastic anemia, which is normally caused by a
deficiency of folate or vitamin B12, not blood
Answer E is incorrect. Vitamin B12 deficiency
loss.
presents as a macrocytic anemia. Peripheral
blood smear typically shows macro-ovalocytosis Answer E is incorrect. Pernicious anemia is
of RBCs. caused by B12 deficiency and presents with a
macrocytic, megaloblastic anemia. The low
Answer F is incorrect. A positive anti-intrinsic
mean corpuscular volume is not consistent
factor antibody would suggest autoimmune de-
with megaloblastic anemia.
struction of parietal cells in the stomach lead-
ing to decreased intrinsic factor and vitamin
18. The correct answer is C. Use of tamoxifen and
HIGH-YIELD SYSTEMS

B12 absorption.
raloxifene as prophylaxis in women at high risk
Answer G is incorrect. In the Schilling test, ra- for breast cancer has been shown to reduce the
dioactive cobalamin is given orally with a non- incidence of breast cancer by 50%–80% in this
radioactive parenteral load. Radioactivity mea- population. It is thought that selective estrogen
sured in the urine can be used to estimate oral receptor modulators have a selective benefit in
cobalamin absorption. A positive Schilling test reducing the incidence of estrogen receptor-
would suggest a defect in absorption. positive cancers for BRCA2-positive women.
The decision to begin tamoxifen and ralox-
17. The correct answer is B. This patient is suffering ifene, however, is not one that should be taken
from iron deficiency anemia secondary to blood lightly. These drugs have many adverse effects
loss from the gastrointestinal tract. Since the that impact quality of life, such as hot sweats
body possesses a limited ability to absorb iron and amenorrhea associated with early meno-
from the gut (which occurs mainly in the duode- pause and increased risk of stroke and pulmo-
num), occult blood loss can quickly lead to a de- nary embolism. Women who have previously
pletion of existing iron stores and hence to an been diagnosed with breast cancer are also of-
Hematology-Oncology

iron deficiency anemia. The burning epigastric ten put on tamoxifen as prophylaxis against a
pain that worsens after eating is consistent with a second cancer.
gastric ulcer. The black stool is melena, which
Answer A is incorrect. Abciximab is an anti-
occurs with blood loss in the upper gastrointesti-
platelet drug used to treat acute coronary syn-
nal tract (bleeding in the lower gastrointestinal
dromes. Aspirin is an irreversible inhibitor of cy-
tract causes visibly bloody red stools). Iron defi-
clooxygenase often used in the treatment and
ciency anemia is shown by the low hematocrit
secondary prevention of acute coronary syn-
level and mean corpuscular volume. The most
dromes.
common cause of iron deficiency anemia in
postmenopausal women is occult blood loss, of- Answer B is incorrect. Flutamide is a non-
ten from a gastrointestinal source. steroidal antiandrogen that competitively in-
hibits androgens at the testosterone receptor.
Answer A is incorrect. Anemia of chronic dis-
Gonadotropin-releasing hormone analogs such
ease is typically a normocytic anemia due to a
as leuprolide are used in continuous dosing
chronic inflammatory, infectious, malignant,
regimens in the treatment of prostate cancer.
or autoimmune disease. This patient’s anemia
is microcytic and is likely due to a condition Answer D is incorrect. Testosterone is an an-
involving blood loss. drogen that is approved by the Food and Drug
Administration for palliation therapy in meta-
Answer C is incorrect. Although this patient’s
static breast cancer. Adverse effects of testoster-
lab values are consistent with iron deficiency
one treatment include virilization, edema, and
anemia, pernicious anemia results instead in
jaundice. Prednisone is a systemic corticoster-
megaloblastic anemia.
oid that is used in various anticancer treatment
 Chapter 11: Hematology-Oncology • Answers 265

regimens. The combination of testosterone to have a high prevalence of the disease and
and prednisone is not used as prophylaxis treat- may be reservoirs for humans. On microscopy,
ment for women at high risk for breast cancer. one observes teardrop-shaped trophozoites with
a ventral sucking disc or cysts with “falling
Answer E is incorrect. Vincristine and vinblas-
leaf” motility. Metronidazole is used to treat G.
tine are microtubule inhibitors used to treat
lamblia infection.
testicular carcinoma as well as Hodgkin’s and
non-Hodgkin’s lymphomas. Answer E is incorrect. Plasmodium ovale is a
form of malaria carried by the Anopheles mos-
19. The correct answer is D. Leishmania donovani quito that causes a 48-hour cyclic fever. Like
infection presents with hepatomegaly and sple- other forms of malaria, it presents as a febrile
nomegaly, malaise, anemia, leukopenia, and illness with fevers accompanied by headaches,
weight loss. L. donovani is transmitted via the sweats, malaise, and anemia (due to lysed

HIGH-YIELD SYSTEMS
sandfly. Microscopically, macrophages contain- RBCs). A feature unique to both P. ovale and
ing amastigotes are observed. Sodium stiboglu- P. vivax is that they can form hypnozoites that
conate is used to treat L. donovani infection. can remain dormant in the liver for long peri-
Answer A is incorrect. Babesia species infec- ods, only to resurface later. Thus after the sys-
tion presents with a malaria-like syndrome. temic infection is cleared, these forms must be
Babesiosis is transmitted by the Ixodes tick and treated with primaquine to kill any organisms
is found in the same regions as Lyme disease. remaining in the liver. P. ovale infection can
On microscopic examination, one observes no be treated with chloroquine.
red blood cell pigment and the Maltese cross-
20. The correct answer is D. The question stem
appearing parasite. Quinine is used to treat
describes follicular lymphoma, the most com-
babesiosis.
mon type of non-Hodgkin’s lymphoma in the
Answer B is incorrect. Entamoeba histolytica United States. The characteristic chromosomal
infection presents with intestinal amebiasis translocation is t(14:18), which juxtaposes the
(bloody diarrhea and abdominal pain) and less IgH locus on chromosome 14 next to the

Hematology-Oncology
commonly liver abscesses. Patients are infected BCL2 locus on chromosome 18. This causes
by ingestion of amebic cysts, usually from con- overproduction of the bcl-2 protein, an anti-
taminated food or water. These cysts pass apoptotic factor, facilitating the survival of the
through the small intestine and form trophozo- cancer. An important simplifying fact to help
ites, which can invade the mucosa of the co- remember the different chromosomal translo-
lon. Microscopy will demonstrate cysts or tro- cations is that those involving the immuno-
phozoites in the stool. Symptomatic patients globulin loci on chromosome 14 tend to be
should be treated with metronidazole. cells that normally produce antibodies (e.g., B
Answer C is incorrect. Giardia lamblia infec- lymphocytes). Thus, these translocations are
tion presents with bloating, flatulence, foul- common in B-lymphocyte lymphomas.
smelling diarrhea, and light-colored fatty stools. Answer A is incorrect. The t(8:14) rearrange-
Abdominal pain, nausea, and vomiting may ment is most commonly found in Burkitt’s
also be present, although in endemic regions lymphoma as well as in some cases of acute
two-thirds of infected individuals are asymp- lymphocytic leukemia. Translocation of the
tomatic. G. lamblia is transmitted via cysts in c-myc gene next to the immunoglobulin heavy
water (fecal-oral transmission) and is common chain locus results in constitutive overproduc-
in all areas of the world, but more prevalent in tion of the c-myc oncogene, promoting neo-
developing countries (although one should plastic proliferation.
consider Giardia infection in patients who
Answer B is incorrect. The t(9:22) transloca-
present with symptoms after camping trips).
tion results in the Philadelphia chromosome,
Domestic cats and dogs, along with beavers
and other mammals, have recently been shown
 266 Section II: Organ Systems • Answers

which is most commonly found in chronic similar results with amoxicillin and erythromy-
myelogenous leukemia as well as in other cin in the treatment of bacterial sinusitis. Con-
chronic myeloproliferative disorders and acute current use of erythromycin and cyclosporine
lymphocytic leukemia, where it confers a poor could lead to elevated cyclosporine serum con-
prognosis. The translocation results in a bcr-abl centrations because erythromycin inhibits the
fusion protein that functions as a constitutively cytochrome P-450 enzyme system in the liver.
active tyrosine kinase to promote leukemia The cytochrome P-450 enzyme superfamily is a
growth. major site of drug metabolism and is responsible
for the oxidation, reduction, and hydrolysis of
Answer C is incorrect. The t(11:22) chromo-
drug compounds, including cyclosporine.
somal translocation is associated with Ewing’s
sarcoma. (Note that it does not involve the im- Answer A is incorrect. Erythromycin will in-
munoglobulin locus.) It overproduces a chime- crease, not decrease, cyclosporine concentra-
HIGH-YIELD SYSTEMS

ric transcription factor that activates the c-myc tions.

promoter and produces excessive amounts of
Answer B is incorrect. Erythromycin will in-
the EWS-FL-1 protein. Ewing’s sarcoma is a
crease cyclosporine concentrations both ini-
small round cell tumor of the bone usually
tially and over time.
found in the long bones of teenagers. X-ray will
show a lytic tumor with reactive bone depos- Answer C is incorrect. Erythromycin will in-
ited around it in an onion-skin fashion. crease cyclosporine concentrations both ini-
tially and over time.
Answer E is incorrect. The t(15:17) transloca-
tion denotes the acute promyelocytic leukemia Answer E is incorrect. Erythromycin will in-
(APL) subtype of acute myelogenous leukemia. crease cyclosporine concentrations.
The characteristic fusion of the promyelocytic
leukemia (PML) gene with the retinoic acid 22. The correct answer is B. Transitional cell car-
receptor-α (RAR-α) gene blocks differentiation cinoma (TCC) is a malignant tumor that arises
of immature myeloid blasts, most likely by from the transitional epithelium of the renal
Hematology-Oncology

blocking activity of other retinoic acid receptors. pelvis. It can spread to adjacent tissues and of-
Treatment with all-trans-retinoic acid (termed ten recurs after removal. It is the most com-
differentiation therapy) overwhelms the block- mon tumor of the urinary tract system. While
ade of the other retinoic acid receptors, restores it can occur in renal calyces, the renal pelvis,
differentiation, and can induce temporary re- and the ureters, the most common site for
mission. Combination differentiation treatment TCC is in the bladder. There is significant as-
together with conventional chemotherapy can sociation of TCC with phenacetin (a common
result in long-term survival rates of 70%–80%, painkiller), smoking, aniline dyes, and cyclo-
unique among the acute leukemias. APL pa- phosphamide (a chemotherapeutic drug); re-
tients also typically present with dysfunctional member the mnemonic “associated problems
coagulopathies, predisposing them to excess in your Pee SAC.” Painless hematuria is the
bleeding, a major source of mortality. most common presenting sign of TCC. The
presence of otherwise unexplained hematuria
21. The correct answer is D. Cyclosporine is an denotes cancer in the urinary tract in individu-
immunosuppressant that binds to cyclophilins, als over the age of 40 years until proven other-
thereby blocking the differentiation and activa- wise. Commonly, TCC is treated surgically.
tion of T lymphocytes by inhibiting the produc- Other treatment modalities include radiation
tion of interleukin-2 (IL-2). Erythromycin is a and chemotherapy as either adjuvant or pri-
macrolide antibiotic that binds to the 50S ribo- mary treatment, depending on the case.
somal subunit and inhibits protein synthesis. Answer A is incorrect. Alcohol use is not a risk
Amoxicillin is a β-lactam antibiotic that inhibits factor associated with transitional cell carci-
bacterial cell wall synthesis. Studies have shown
 Chapter 11: Hematology-Oncology • Answers 267

noma, but is associated with many other can- factor to form intrinsic factor/vitamin B12 com-
cers, including esophageal and liver cancers. plexes.
Answer C is incorrect. Exposure to asbestos is Answer E is incorrect. Pepsin, which is active
not a risk factor associated with transitional cell in creating the acidic pH of the stomach, splits
carcinoma, but is associated with an increased vitamin B12 from its exogenous protein-bound
risk for developing mesothelioma. form, leaving it free to bind R proteins pro-
duced by the salivary gland.
Answer D is incorrect. Hypertension is not a
risk factor associated with transitional cell car-
24. The correct answer is B. Wilms’ tumor arises
cinoma.
from neoplastic embryonal renal cells of the
Answer E is incorrect. Previous pyelonephritis metanephros. Wilms’ tumor is the most com-
is not a risk factor associated with transitional mon solid tumor of childhood (most com-

HIGH-YIELD SYSTEMS
cell carcinoma. monly occurring between the ages of 2 and 4
years) and is rarely seen in adults. It commonly
23. The correct answer is C. The clinical and lab- presents with a huge palpable flank mass and is
oratory findings are consistent with pernicious seen with hemihypertrophy (abnormal enlarge-
anemia. This chronic progressive anemia is ment of one side of the body). It is associated
caused by a deficiency of intrinsic factor, a pro- with the deletion of tumor suppressor gene
tein produced by gastric parietal cells. Once vi- WT1 on chromosome 11. Since it arises from
tamin B12 is released from food, it binds R-pro- the kidney parenchyma, it distorts the kidney
teins upon which it travels to the duodenum. calyces as it grows.
In the duodenum, vitamin B12 is released from
Answer A is incorrect. Adult polycystic disease
R-proteins and then complexes with intrinsic
is an autosomal dominant genetic disorder that
factor. The intrinsic factor-B12 complex binds
presents with bilateral cystic enlargement of
to receptors in the ileum, where it is endocy-
the kidneys. Individuals with this disorder also
tosed by enterocytes. Pernicious anemia is be-
suffer from cystic enlargement of the liver,
lieved to be caused by immune-mediated de-

Hematology-Oncology
berry aneurysms, and mitral valve prolapse.
struction of gastric mucosa, leading to a loss of
parietal cells. A Schilling test (as described in Answer C is incorrect. Clear cell carcinoma of
the stem) will reveal whether the labeled vita- the kidney is a malignancy derived from the re-
min B12 is being absorbed and eliminated in nal tubular cells. It is common for patients to
the urine (as in normal individuals), or whether present with an abdominal mass, but patients
a lack of intrinsic factor or malabsorption is with clear cell carcinoma are commonly men
preventing the radiolabeled compound from 50–70 years old, with an increased incidence
appearing in the urine. found in smokers. Patients with renal cell carci-
noma present with a range of symptoms, such
Answer A is incorrect. Transcobalamin II, a
as hematuria, a palpable mass, polycythemia,
plasma protein, binds free vitamin B12 in ileal
flank pain, and fever.
cells for transport through the bloodstream.
Answer D is incorrect. TCC is a malignant tu-
Answer B is incorrect. R proteins are vitamin
mor that arises from the uroepithelial cells of
B12-binding proteins produced by the salivary
the urinary tract. TCC is the most common tu-
gland that bind vitamin B12 once it has been
mor of the urinary tract (as it can occur in the
split from its exogenous protein-bound form by
renal calyx, renal pelvis, ureters, and bladder).
pepsin in the stomach.
Painless hematuria and urinary outflow ob-
Answer D is incorrect. Pancreatic proteases struction are the most common presenting
are secreted into the duodenum, which split signs of TCC.
the R-protein/vitamin B12 complexes, produc-
ing vitamin B12 that is free to bind intrinsic
 268 Section II: Organ Systems • Answers

Answer E is incorrect. Malignancy that is de- thrombus formation in the microvasculature,

rived from the primitive neural crest cells as an evidence of intravascular hemolysis is found
abdominal mass in a young child is neuroblas- due to microangiopathy. The classic pentad of
toma. The tumor does not arise in the kidney; TTP consists of fever, thrombocytopenia, mi-
instead, it forms from the adrenal medulla and croangiopathic hemolysis, neurologic symp-
paraspinal sympathetic ganglia. Therefore, it toms, and renal insufficiency. RBC lysis is
does not distort the kidney architecture. caused by shear trauma in the vessels and is
not secondary to complement-mediated lysis.
25. The correct answer is D. This patient suffers
from paroxysmal nocturnal hemoglobinuria, 26. The correct answer is B. Cisplatin has been
an acquired disease caused by RBC susceptibil- shown to cause nephrotoxicity and acoustic
ity to complement-mediated cell lysis. This is nerve damage. Use of cisplatin requires that the
HIGH-YIELD SYSTEMS

due to insufficient synthesis of GPI anchors, patient be vigorously hydrated in order to pre-
which anchor proteins that protect RBCs from vent kidney damage. In patients with docu-
this process. Clinical manifestations include mented kidney dysfunction, cisplatin should be
intravascular hemolysis with hemoglobin re- avoided altogether due to these patients’ greater
lease into the blood and subsequent hemoglo- susceptibility to dose-related nephrotoxicity.
binuria, thrombotic complications (such as
Answer A is incorrect. Bleomycin toxicity in-
Budd-Chiari syndrome), and aplastic anemia.
cludes pulmonary fibrosis, hypertrophic skin
Answer A is incorrect. Autoimmune hemolytic changes, and minimal myelosuppression. It is
anemia is caused by IgG antibodies that are not associated with nephrotoxicity.
targeted against RBC antigens. This patient’s
Answer C is incorrect. Cytarabine causes leu-
symptoms are all due to acute intravascular
kopenia, thrombocytopenia, and megaloblastic
hemolysis. In this condition the hemolysis is
anemia. It is not associated with nephrotoxic-
extravascular and antibody-mediated and is not
ity.
due to complement-mediated lysis.
Answer D is incorrect. Toxic effects of doxoru-
Hematology-Oncology

Answer B is incorrect. Although common bile

bicin include cardiotoxicity, myelosuppression,
duct stricture may cause darker urine due to
and alopecia. It is not associated with nephro-
elevated bilirubin in the blood, it would not
toxicity.
cause intravascular hemolysis or complement-
mediated lysis. Common bile duct strictures Answer E is incorrect. Tamoxifen has been
caused by carcinomas of the pancreatic head noted to cause hot flashes, nausea/vomiting,
are the most common causes of painless jaun- skin rash, and vaginal bleeding. It is not associ-
dice. ated with nephrotoxicity.
Answer C is incorrect. Hereditary spherocyto- 27. The correct answer is A. This patient presents
sis is caused by mutations that result in an un- with clinical features of idiopathic thrombocy-
stable erythrocyte cytoskeleton. When those topenic purpura (ITP), the most common
erythrocytes pass through the spleen, the retic- cause of thrombocytopenia in childhood. ITP
uloendothelial cells remove pieces of the mem- is an autoimmune disease and is most com-
brane, causing spherocyte formation. This re- monly instigated by a viral illness. Bleeding dis-
sults in extravascular hemolysis and is not due orders due to platelet defects or deficiencies
to complement-mediated lysis. will present with microhemorrhage of the mu-
Answer E is incorrect. The normal von Wille- cous membranes and of the skin, like the non-
brand factor (vWF) protease is absent in pa- blanching purple skin lesions (purpura) seen
tients with thrombotic thrombocytopenic pur- in this patient. ITP is a diagnosis of exclusion
pura (TTP), resulting in high levels of large but classically presents with thrombocytopenia,
vWF multimers and causing excessive platelet antiplatelet antibodies, as well as signs of a
adhesion and clearance. Because of diffuse compensatory increase in platelet production,
 Chapter 11: Hematology-Oncology • Answers 269

including large platelets on peripheral blood duction has lead to increased glucocorticoids.
smear and increased megakaryocytes on bone Weight gain and redistribution of body fat (in
marrow biopsy. As ITP only affects platelets, contrast to the cachexia typical of cancers
patients will have normal prothrombin time alone) and moon facies are classic signs of
and normal partial thromboplastin time. Cushing’s syndrome. Poor wound healing (due
to inhibition of collagen synthesis by glucocor-
Answer B is incorrect. Decreased megakaryo-
ticoids) and hirsutism (stimulation of androgen
cytes on bone marrow biopsy suggest a defect
production by ACTH) are also part of Cush-
in platelet production which can result from
ing’s syndrome.
malignancy, infection, or drug reactions.
Answer A is incorrect. Cold intolerance and
Answer C is incorrect. Patients with IgA defi-
hair loss are symptoms of hypothyroidism.
ciency present with frequent respiratory, uri-

HIGH-YIELD SYSTEMS
nary, and gastrointestinal infections and would Answer B is incorrect. Galactorrhea and
not present with isolated thrombocytopenia. amenorrhea are symptoms of hyperprolactine-
mia, as may occur in cases of anterior pituitary
Answer D is incorrect. Increased fibrin split
tumors.
products are a sign of activation of the coagula-
tion cascade and may be associated with Answer C is incorrect. Insatiable thirst and
thrombocytopenia in the case of DIC. DIC is polyuria are symptoms of ADH deficiency or
caused by a systemic activation of the clotting diabetes insipidus.
cascade leading to global depletion of clotting
Answer E is incorrect. Restlessness and tremor
factors in addition to depletion of platelets.
are symptoms of hyperthyroidism.
DIC leads to complications from both the sys-
temic microthrombi and the increased risk of 29. The correct answer is A. This woman suffers
bleeding from clotting factor and platelet de- from hereditary spherocytosis (HS), typically
pletion. While patients with DIC will present caused by mutations in the genes that code for
with thrombocytopenia, they will also have an either ankyrin or spectrin. Both of these pro-
increased prothrombin time and partial throm-

Hematology-Oncology
teins contribute to the erythrocyte cytoskeleton.
boplastic time and schistocytes on their periph- When the erythrocytes with abnormal mem-
eral blood smears. In addition to the microhe- branes pass through the spleen, the reticuloen-
morrhage of the skin and mucous membranes dothelial cells remove pieces of the membrane,
due to the platelet deficiency, patients with causing a decreased membrane-to-cytoplasm
DIC can also develop macrohemorrhage in ratio. This results in spherocyte formation. HS
joints and internal organs as a result of the clot- is often associated with hemolytic crisis result-
ting factor deficiencies. ing in jaundice and pigmented gallstones.
Answer E is incorrect. Vitamin K deficiency Answer B is incorrect. Glucose 6-phosphate
leads to a deficiency in the Vitamin K-dependent dehydrogenase (G6PD) deficiency is due to an
clotting factors, II, VII, IX, and X, causing an ele- X-linked mutation and therefore typically oc-
vated prothrombin time and increasing the risk curs only in males. Although hemolysis in the
of macrohemorrhage. Vitamin K deficiency, setting of G6PD could be secondary to infec-
however, should not lead to thrombocytopenia tion, Heinz bodies and bite cells will be found
seen in this patient. in the peripheral blood smear, not spherocytes.
28. The correct answer is D. Although her symp- Answer C is incorrect. Autoimmune hemo-
toms may be somewhat nonspecific, the fact lytic anemia is due to antibodies against eryth-
that this patient has small-cell lung carcinoma rocytes. This will cause a positive Coombs’ test
(SCLC) should be a clue that her symptoms and therefore is unlikely in this setting.
may be part of a paraneoplastic syndrome. Answer D is incorrect. Iron deficiency anemia
SCLC is notorious for production of ACTH causes a microcytic anemia. This does not fit
and ADH. In this case, excessive ACTH pro-
 270 Section II: Organ Systems • Answers

the hemolytic picture and is not associated community are already known, only newborn
with spherocytes. infants are truly at risk for sickle cell disease,
and thus the denominator in the calculation of
Answer E is incorrect. Hemolysis because of a
incidence would be 100, not 6020.
mechanical valve is caused by mechanical
trauma as the erythrocytes flow across the for- Answer E is incorrect. This answer fails to take
eign surface. This results in schistocytes in a into account the deaths that occurred during
peripheral blood smear. This diagnosis is un- the year, which would affect both the total
likely given the absence of a unique heart number of current cases and the current popu-
sound on examination and an absence of schis- lation.
tocytes in the smear.
31. The correct answer is D. This bone marrow
30. The correct answer is D. Prevalence is the to- biopsy is consistent with multiple myeloma.
HIGH-YIELD SYSTEMS

tal number of cases in a population divided by Plasma cells can be clearly seen throughout
the total population; that is, the proportion of this image, recognized by their off-center nu-
the total population with the disease. The total clei and clock-face chromatin distribution. Also
number of cases at the beginning of the study commonly seen on a blood smear are stacked
can be calculated by multiplying the initial RBCs, in what is known as a rouleaux forma-
population (6000) by the initial prevalence tion.
(10%), yielding 600 cases. Over the course of
Answer A is incorrect. Bone marrow suppres-
the year, there was a net gain of 3 patients with
sion appears as a paucity of cells on histology.
sickle cell disease (6 births – 3 deaths), bring-
This image has too many plasma cells to repre-
ing the new total to 603. Likewise, the new to-
sent bone marrow suppression.
tal population is 6020, a net gain of 20 people.
Therefore, the current prevalence of sickle cell Answer B is incorrect. Lymphoma is a neo-
disease is 603/6020. plastic disorder of the lymphoid tissue. There
are many different types of lymphoma, two of
Answer A is incorrect. Prevalence is the total
the most distinctive histologic types being
Hematology-Oncology

number of cases in a population divided by the

Burkitt’s lymphoma and Hodgkin’s lymphoma.
total population. The value in this answer
Burkitt’s lymphoma shows a “starry sky” pat-
choice describes the net gain in the number of
tern on histology. This pattern is created by
cases divided by the population, and therefore
macrophage ingestion of tumor cells. Hodg-
is incorrect.
kin’s lymphoma is distinguished by its Reed-
Answer B is incorrect. Prevalence is com- Sternberg cells. The Reed-Sternberg cells are
monly confused with incidence, which is the binucleate and display prominent nucleoli.
number of new cases in a unit of time divided
Answer C is incorrect. Prostate cancer is typi-
by the number of susceptible people. This an-
cally adenocarcinoma. It commonly metasta-
swer choice represents the incidence of sickle
sizes to the axial skeleton and can cause back
cell disease during the year, not the preva-
pain and spinal cord compression. It causes os-
lence. Because the genotype of all community
teoblastic lesions in bone.
members is already known, only newborn in-
fants are truly at risk for the disease. Therefore, Answer E is incorrect. This image shows too
of the 100 people at risk for sickle cell disease, many plasma cells to represent normal bone
6 were diagnosed, yielding an incidence of marrow.
6/100.
32. The correct answer is E. Trimethoprim is as-
Answer C is incorrect. This value would rep- sociated with megaloblastic anemia, leukope-
resent the incidence of the disease if the entire nia, and granulocytopenia. These effects may
population were at risk. However, because the be reduced with a folic acid supplement.
genotypes of all preexisting members of the
 Chapter 11: Hematology-Oncology • Answers 271

Answer A is incorrect. Chloramphenicol is a tension, increased appetite, elevated blood

broad-spectrum antibiotic that is particularly sugar, indigestion, insomnia, nervousness, rest-
effective against bacterial meningitis and is as- lessness, and immunosuppression. However, in
sociated with aplastic anemia and gray baby addition, prednisone is known to produce pro-
syndrome. found mood changes known as glucocorticoid
psychosis.
Answer B is incorrect. Clindamycin is associ-
ated with pseudomembranous colitis due to Answer A is incorrect. The typical adverse ef-
Clostridium difficile overgrowth. fects of bleomycin are pulmonary fibrosis, skin
changes, and myelosuppression. Bleomycin is
Answer C is incorrect. Erythromycin is a mac-
part of the ABVD cancer chemotherapy regi-
rolide and is associated with cholestatic hepati-
men against Hodgkin’s: Adriamycin (doxorubi-
tis.
cin), Bleomycin, Vinblastine, and Dacarba-

HIGH-YIELD SYSTEMS
Answer D is incorrect. Gatifloxacin is a fluoro- zine.
quinolone and is associated with tendinitis.
Answer C is incorrect. Common adverse ef-
33. The correct answer is B. Rectal bleeding or fects of vincristine are areflexia and peripheral
anemia in anyone age 50 years or older is con- neuritis. Vincristine is part of the MOPP can-
sidered colorectal cancer until proven other- cer chemotherapy regimen used against Hodg-
wise. The gold standard for diagnosis of col- kin’s disease: Mechlorethamine, vincristine
orectal cancer is colonoscopy with possible (Oncovin), Procarbazine, and Prednisone.
tissue biopsy of suspicious lesions. Current Answer D is incorrect. Wildly swinging mood
cancer screening recommendations suggest is suggestive of cyclothymic disorders, which
colonoscopy or double-contrast barium enema are common in patients with chronic medical
every 10 years, sigmoidoscopy every 5 years, illness. However, given that this disorder re-
and/or fecal occult blood testing in people ≥50 quires 2 years of symptoms before a definitive
years old. diagnosis can be made, the most likely cause is
glucocorticoid psychosis.

Hematology-Oncology
Answer A is incorrect. Abdominal plain films
are best used to visualize radiopaque renal lith- Answer E is incorrect. The progression of
iasis, free air, or fluid. Hodgkin’s disease typically does not involve
Answer C is incorrect. Abdominal CT scans profound psychiatric symptoms.
can visualize many abdominal pathologies;
35. The correct answer is A. The patient has a hy-
however, colonoscopy is a more sensitive test
datidiform mole. Hydatidiform moles are cystic
for colorectal cancer screening.
swellings of the chorionic villi. They usually
Answer D is incorrect. Esophagoduodeno- present in the fourth and fifth months of preg-
scopy is best used to evaluate for lesions of the nancy with vaginal bleeding. On exam the
upper gastrointestinal tract. uterus is larger than expected for gestational
Answer E is incorrect. Pelvic ultrasound is best age and the serum β-hCG level is much higher
used to evaluate abnormalities of the ovaries, than normal. Moles can be either partial or
uterus, and vagina. complete and are caused by either fertilization
of an egg that has lost its chromosomes or fer-
34. The correct answer is B. This patient is pre- tilization of a normal egg with two sperm. Par-
senting with some of the classic adverse effects tial moles may contain some fetal tissue but no
of steroid therapy, which is often part of treat- viable fetus, and a complete mole contains no
ment for Hodgkin’s disease. These include the fetal tissue. Hydatidiform moles must be surgi-
physical signs of Cushing’s syndrome (weight cally removed because the chorionic villi may
gain, moon facies, thin skin, muscle weakness, embolize to distant sites and because moles
and brittle bones), along with cataracts, hyper- may lead to choriocarcinoma, an aggressive
 272 Section II: Organ Systems • Answers

neoplasm that metastasizes early but is very re- usually does not have negative effects on the
sponsive to chemotherapy. fetus because β-chains are not expressed until
after birth.
Answer B is incorrect. Coma is a possible out-
come of eclampsia, not of a hydatidiform Answer B is incorrect. There is no excess of
mole. Preeclampsia is the triad of hyperten- α-chains in α-thalassemia
sion, proteinuria, and edema. Eclampsia oc-
Answer C is incorrect. Excess β-globin chains
curs when seizures accompany the symptoms
would be seen in the case of hemoglobin H
of preeclampsia. This patient does not have
(HbH) disease (another type of α-thalassemia),
any of these symptoms.
in which three α-globin chains have been de-
Answer C is incorrect. Neural tube defects are leted. Here, β-globin chains make tetramers
usually detected in utero by increased α-feto- known as HbH. HbH does have a higher affin-
HIGH-YIELD SYSTEMS

protein levels in amniotic fluid and maternal ity for oxygen, and there is usually tissue hy-
serum; β-hCG levels are normal in these pa- poxia associated with this disease. However, hy-
tients. drops fetalis is not associated with this
condition because the hypoxia occurs in the
Answer D is incorrect. Ovarian cancers are of-
adult when β-chains are expressed.
ten accompanied by an increase in blood can-
cer antigen 125 levels, not β-hCG levels. This Answer D is incorrect. Excess β-globin chains
patient has a hydatidiform mole, not ovarian leading to hemoglobin H actually bind more
cancer. Hydatidiform moles do not predispose strongly to oxygen.
patients to ovarian cancer.
Answer F is incorrect. In hemoglobin Barts,
Answer E is incorrect. If undetected, tubal the hemoglobin binds more strongly to oxygen,
pregnancies may rupture, causing unilateral not more weakly.
lower quadrant pain and uterine bleeding. Be-
cause the patient may exsanguinate, ruptured 37. The correct answer is F. This vignette suggests
ectopic pregnancy is a surgical emergency. a malignancy of the liver. Hepatomas are
Hematology-Oncology

This patient does not have the symptoms of a highly associated with chronic hepatitis B and
tubal pregnancy. Also, because of the small C infections, which are often found in health
size of the fallopian tubes, tubal pregnancies care workers due to needle stick injuries. Other
present long before 4 months of gestation. risk factors for hepatomas include Wilson’s dis-
ease, hemochromatosis, alcoholic cirrhosis,
36. The correct answer is E. When the α-globin α-1-antitrypsin deficiency, and carcinogens.
genes are missing (as in the most severe type of α-Fetoprotein is a marker for hepatomas but
α-thalassemia), excess gamma-globin chains can also be elevated in patients with germ cell
accumulate, leading to the formation of tetram- tumors, such as yolk sac tumors. Tumor mark-
ers known as hemoglobin Barts. These tetram- ers should not be used for primary diagnoses,
ers bind so strongly to oxygen that the fetal but for confirmation and to monitor therapy.
tissues are not oxygenated properly. This severe
Answer A is incorrect. The marker for chorio-
tissue anoxia leads to hydrops fetalis, an abnor-
carcinoma is β-human chorionic gonadotro-
mal fluid accumulation in at least 2 fetal com-
pin. This marker is also elevated with hydatidi-
partments.
form moles and gestational trophoblastic
Answer A is incorrect. α-Thalassemia occurs tumors. The presence of a hepatoma has no ef-
when one or more α-globin genes is deleted, fect on this marker.
leading to accumulation of other globin chains.
Answer B is incorrect. The marker for colorec-
For this reason, one would not expect to see ac-
tal carcinoma is carcinoembryonic antigen.
cumulation of α-globin chains in α-thalassemia.
This marker is nonspecific and is also pro-
Excess α-globin chains would be expected in
duced by pancreatic, gastric, and breast
β-thalassemia minor or major. β-Thalassemia
 Chapter 11: Hematology-Oncology • Answers 273

carcinomas. The presence of a hepatoma has 39. The correct answer is D. The testes begin life
no effect on this marker. high in the abdomen and descend to their final
resting place in the scrotum. The lymphatic
Answer C is incorrect. The marker for mela-
drainage from the testes, therefore, is to the
noma is S-100. This marker also is elevated
para-aortic lymph nodes in the lumbar region
with neural tumors and astrocytomas. The
just inferior to the renal arteries.
presence of a hepatoma has no effect on this
marker. Answer A is incorrect. The deep inguinal
nodes drain the vessels in the spongy urethra
Answer D is incorrect. The marker for neuro-
and may become enlarged in some sexually
blastoma is bombesin. This marker also is ele-
transmitted diseases or other causes of urethri-
vated with lung and gastric cancers. The pres-
tis.
ence of a hepatoma has no effect on this

HIGH-YIELD SYSTEMS
marker. Answer B is incorrect. External iliac nodes
drain the bladder.
Answer E is incorrect. The marker for pros-
tatic carcinoma is prostate-specific antigen. Answer C is incorrect. Gluteal lymph nodes
The presence of a hepatoma has no effect on drain the deep tissue of the buttocks.
this marker.
Answer E is incorrect. Tumors of the scrotum
itself, but not of the testes, may spread to the
38. The correct answer is A. Neoplastic B lympho-
superficial inguinal lymph nodes. The scrotum
cytes are the cells of origin in most non-Hodg-
is an outpouching of abdominal skin, and
kin’s lymphomas (90% of cases), with the nota-
drainage of this skin is to the superficial ingui-
ble exception of lymphoblastic lymphoma,
nal nodes. The testes, however, which lie in-
which is typically dominated by T lymphocytes.
side the scrotum, begin life in the abdomen,
Answer B is incorrect. Myeloblasts are the and lymph drainage follows embryologic ori-
neoplastic cells in acute myelogenous leuke- gins.
mia.

Hematology-Oncology
40. The correct answer is A. This woman suffers
Answer C is incorrect. Plasma cells are the
from von Willebrand’s disease, the most com-
neoplastic cells in multiple myeloma. Multiple
mon inherited bleeding disorder; it results from
myeloma also affects patients in their 50s and
a defective form or overall deficiency of vWF.
60s. However, at presentation patients with
vWF has two functions: it serves as the ligand
multiple myeloma usually have pathologic
for platelet adhesion to a damaged vessel wall,
fracture caused by lytic lesions, hypercalcemia
and it also is the plasma carrier of factor VIII.
because of bone resorption, and repeated in-
Due to platelet dysfunction and lack of a car-
fection because of decreased production of
rier for factor VIII, the unique lab finding in
normal immunoglobulins. Urine analysis in
this disease consists of an increased bleeding
patients with multiple myeloma shows Bence
time and an increased partial thromboplastin
Jones proteinuria with a monoclonal spike on
time. Cryoprecipitate is the precipitate that re-
electrophoresis.
mains when fresh frozen plasma is thawed. It
Answer D is incorrect. Reed-Sternberg cells contains sufficient normal vWF to correct the
are the neoplastic cells in Hodgkin’s disease. bleeding dyscrasia. In addition to prolonged
Under light microscopy, Reed-Sternberg cells bleeding from mucosal surfaces as in this pa-
appear as large binucleate cells with abundant tient, other symptoms include easy bleeding
cytoplasm and large “owl-eye” nucleoli. and skin bleeding.
Answer E is incorrect. T- lymphocyte lympho- Answer B is incorrect. Factor VIII concentrate
mas are less common and include lymphoblas- is used to treat individuals with hemophilia A,
tic lymphomas and a minority of diffuse large an inherited condition that results in factor
cell lymphomas. VIII deficiency.
 274 Section II: Organ Systems • Answers

Answer C is incorrect. Fresh frozen plasma fore, a decrease in the levels of fibrinogen
(FFP) is used to treat several factor deficien- would be expected.
cies, including V, VII, X, and XI. FFP adminis-
Answer D is incorrect. Because the anticoagu-
tration will replace several factor deficiencies,
lation factors are also being activated and con-
although factor concentrations in FFP tend to
sumed during DIC, protein C levels would de-
vary. Unlike cryoprecipitate, FFP does not
crease.
contain von Willebrand factor or fibrinogen.
FFP may be needed for inherited factor XI de- Answer E is incorrect. Because anticoagula-
ficiency or as a source of factor V in severe tion factors are being consumed during DIC,
cases of disseminated intravascular coagulation protein S levels would decrease.
when platelet concentrates and cryoprecipitate
do not correct the factor V, VIII, and fibrino- 42. The correct answer is E. The patient has a pro-
longed prothrombin time, likely indicating a
HIGH-YIELD SYSTEMS

gen consumption defects.

deficiency in one of the factors involved with
Answer D is incorrect. LMWH is an anticoag- the extrinsic pathway. Vitamin K is a fat-soluble
ulant that acts predominantly on factor Xa. This vitamin that is a cofactor for the γ-carboxylation
patient is in need of a procoagulant rather than of glutamate residues of prothrombin; factors
an anticoagulant. LMWH can be administered VII, IX, and X; and proteins C and S. Vitamin
subcutaneously. One advantage of LMWH K deficiency is uncommon; however, it can oc-
over heparin is that the partial thromboplastin cur in the setting of oral broad-spectrum antibi-
time does not need to be routinely monitored otics, which suppress the flora of the bowel and
with this drug. interfere with the absorption and synthesis of
Answer E is incorrect. Protamine sulfate is this vitamin. It can also be associated with other
used for reversal of heparinization. It is a posi- conditions related to fat malabsorption and dif-
tively charged molecule that acts by binding fuse liver disease, or in the neonatal period
to heparin, a negatively charged molecule. when the intestinal flora have not developed
Protamine would have no therapeutic benefit and the liver reserves of vitamin K are small.
Hematology-Oncology

for this patient. Vitamin K deficiency usually presents with

bleeding diathesis, hematuria, melena, bleed-
41. The correct answer is A. DIC can occur in the ing gums, and ecchymoses.
setting of obstetric complications, sepsis, ma-
Answer A is incorrect. The activity of factor
lignancy, and other conditions. It is described
VIII does not depend on vitamin K.
as a thrombohemorrhagic process because
there are microthrombi throughout the body, Answer B is incorrect. The activities of factors
and coagulation factors and platelets are con- VIII and XII do not depend on vitamin K.
sumed actively. The active conversion of fi- Answer C is incorrect. The activities of factors
brinogen to fibrin as part of the convergence of IX and XII do not depend on vitamin K.
both clotting cascades leads to decreased levels
of fibrinogen. At the same time, anticoagula- Answer D is incorrect. The activity of factor
tion factors such as plasmin and protein C are XII does not depend on vitamin K.
being activated, leading to fibrinolysis and in-
creased levels of D-dimers in the circulation. 43. The correct answer is D. This patient likely has
glucose 6-phosphate dehydrogenase deficiency,
Answer B is incorrect. DIC leads to consump- which is common in African-Americans, and
tion of coagulation factors; therefore, a drop in demonstrates characteristic Heinz bodies (pre-
factor VII levels would be expected. cipitates of hemoglobin) within RBCs. Hemol-
Answer C is incorrect. Fibrinogen is actively ysis can be precipitated by certain drugs, such
converted to fibrin in the setting of DIC; there- as sulfonamides, isoniazid, aspirin, ibuprofen,
primaquine, and nitrofurantoin.
 Chapter 11: Hematology-Oncology • Answers 275

Answer A is incorrect. Aminoglycosides are dice and a palpable, enlarged, nontender gall-
commonly associated with nephro- and ototox- bladder. This sign is often found in patients
icity. with cancer in the head of the pancreas, which
is obstructing the gallbladder from emptying.
Answer B is incorrect. Fluoroquinolones may
cause some gastrointestinal upset, damage to Answer A is incorrect. Acute hepatitis is associ-
cartilage in children, and tendonitis and ten- ated with tenderness in the right upper quad-
don rupture in adults. rant, and it should not produce an enlarged
gallbladder.
Answer C is incorrect. Macrolide toxicity in-
cludes gastrointestinal discomfort, acute chole- Answer B is incorrect. Choledocholithiasis is
static hepatitis, eosinophilia, and skin rashes. the presence of a stone in the common bile
duct. It is a common cause of cholecystitis,
Answer E is incorrect. Tetracycline toxicity in-

HIGH-YIELD SYSTEMS
which is associated with tenderness in the right
cludes gastrointestinal distress, tooth discolor-
upper quadrant.
ation and inhibition of bone growth in chil-
dren, and photosensitivity reactions. Answer C is incorrect. Cholelithiasis simply
refers to stones within the gallbladder. It is gen-
44. The correct answer is A. Abciximab functions erally painless, and does not cause jaundice or
by binding to the glycoprotein receptor IIb/IIIa an enlarged gallbladder.
on activated platelets, preventing fibrinogen
Answer D is incorrect. Hemolytic anemia is a
from binding and interfering with platelet ag-
cause of painless jaundice, however, it is not
gregation. It is used in acute coronary syn-
associated with an enlarged gallbladder.
drome and angioplasty.
Answer B is incorrect. Both clopidogrel and 46. The correct answer is B. This patient has DIC
ticlopidine function by inhibiting the ADP secondary to an acute case of pancreatitis. Com-
pathway involved in the binding of fibrinogen mon causes of DIC are gram-negative sepsis,
to platelets during platelet aggregation. malignancy, acute pancreatitis, trauma, transfu-

Hematology-Oncology
sion reactions, and obstetric complications. Dur-
Answer C is incorrect. Leuprolide is a gonado-
ing DIC, there is a massive activation of the co-
tropin-releasing hormone analog that acts as an
agulation cascade that results in thrombus
agonist when administered in a pulsatile fashion
formation throughout the microvasculature.
and as an antagonist when administered in a
Rapid consumption of both platelets and coagu-
continuous fashion. It is used to treat infertility
lation factors follows, with subsequent easy
(when administered as an agonist), prostate can-
bleeding and bruising. Concurrent with this
cer (when administered as an antagonist), and
consumptive coagulopathy is activation of the
uterine fibroids.
fibrinolytic system. In this patient, thrombocy-
Answer D is incorrect. Selegiline is a selective topenia, elevated prothrombin time/partial
monoamine oxidase B inhibitor that causes an thromboplastin time, and the presence of fibrin
increase in the availability of dopamine. It is split products all suggest DIC. Pancreatitis as
used with levodopa in the treatment of Parkin- the cause is supported by the clinical presenta-
son’s disease. tion of epigastric pain that may radiate to the
Answer E is incorrect. Both clopidogrel and back, nausea, vomiting, and increased amylase
ticlopidine function by inhibiting the ADP and lipase levels. The presence of a dilated com-
pathway involved in the binding of fibrinogen mon bile duct suggests that gallstones may have
to platelets during platelet aggregation. been the cause of the pancreatitis episode. Dila-
tion of the common bile duct can be seen proxi-
45. The correct answer is E. This patient’s physi- mal to an area of current or past obstruction. An
cal exam demonstrates Courvoisier’s sign: jaun- obstruction in the ampulla of Vater or simply
 276 Section II: Organ Systems • Answers

the passing of a gallstone through the ampulla tion of clotting factors and thrombopoietin is
can block the flow of pancreatic exocrine en- not often impaired. This, combined with the
zymes from the pancreatic duct, and the back- low likelihood of disseminated intravascular
flow of these enzymes into the pancreas leads to coagulation in acute cholecystitis, means that
autodigestion of the pancreatic parenchyma. PT, PTT, and platelet counts should be nor-
This autodigestion leads increased blood levels mal, and there should be no signs of easy
of the pancreatic enzymes amylase and lipase. bleeding or bruising. Also, amylase and lipase
levels should be normal in acute cholecystitis.
Answer A is incorrect. Acute hepatitis is an in-
Ultrasounds would likely show cholelithiasis,
flammatory process leading to liver cell death,
but cholecystitis is not necessarily associated
likely caused by viral agents, drugs, or poisons.
with dilation of the common bile duct since
Signs and symptoms of acute hepatitis vary
the obstruction usually occurs in the cystic
greatly among patients but can range from as-
HIGH-YIELD SYSTEMS

duct.
ymptomatic to fatal. Patients may present with
nausea and vomiting. Easy bruising and bleed- Answer D is incorrect. Gastric ulcers may
ing secondary to decreased hepatic product of present clinically as epigastric pain radiating to
clotting factors may occur, with subsequent the back and may be associated with increased
prolongation to PT and PTT. Thrombocytope- amylase activity, especially if perforation has oc-
nia may occur secondary to decreased hepatic curred. However, ulcers are not typical causes
production of thrombopoietin, with subsequent of disseminated intravascular coagulation or
prolongation of bleeding time. Gallstones, as subsequent findings of thrombocytopenia, in-
suggested by a dilated common bile duct, creased PT and PTT, and fibrin split products.
could cause obstruction of the biliary tree and Furthermore, gastric ulcers will not cause in-
damage the liver. However, unlike the presen- creased amylase and lipase levels and are not
tation in this patient, pain associated with generally related to common bile duct dila-
acute hepatitis is likely to develop in the right tion.
upper abdominal quadrant. Fever may also be
Answer E is incorrect. von Willebrand’s dis-
present, especially with viral etiologies. While
Hematology-Oncology

ease is characterized by defect or deficiency of

aspartate aminotransferase, alanine aminotrans-
vWF. During clot formation in hemostasis,
ferase, and alkaline phosphatase levels are
vWF functions to link platelets to collagen in
commonly elevated in acute hepatitis, amylase
exposed endothelium and serves as a carrier for
and lipase levels are not. Fibrin split products
factor VIII. Symptoms include easy bruising
are unlikely to be present.
and bleeding. Like this patient, people with
Answer C is incorrect. Acute cholecystitis is von Willebrand’s disease are likely afrebrile.
defined as inflammation of the gallbladder Bleeding time would be prolonged due to de-
generally associated with cholelithiasis. Patho- creased platelet adhesion, and PTT would be
genesis typically involves obstruction of the increased due to decreased available VIII (a
cystic duct by gallstones. Acute cholecystitis component of the intrinsic pathway). However,
may present with elevated WBC counts, nau- the sudden onset of disease as an adult makes
sea, and vomiting. However, unlike in this pa- this hereditary disease a less likely cause of this
tient, the pain is more likely to be located in patient’s symptoms. Furthermore, abdominal
the right upper quadrant and may radiate to pain, fever, increased WBC counts, thrombo-
the scapula or shoulder. A low-grade fever is cytopenia, increased PT, fibrin split products,
common. Because the obstruction by gall- and common bile duct dilation would not be
stones is generally restricted to the cystic duct likely findings in von Willebrand’s disease.
and not to the hepatic duct or biliary tree, liver
damage is not common. In fact, total bilirubin 47. The correct answer is D. Monoclonal gammo-
and alkaline phosphatase levels are elevated in pathy of undetermined significance (MGUS)
only a minority of cases, and hepatic produc- is characterized as a plasma cell dyscrasia, de-
 Chapter 11: Hematology-Oncology • Answers 277

fined by the presence of a monoclonal immu- Answer A is incorrect. α-Thalassemia minor is

noglobulin, also called an M protein, in the se- associated with a two-gene deletion of α globin
rum or urine of persons without evidence of (two gene regions are intact). Deletion of only
multiple myeloma, Waldenström’s macroglob- a single α gene results in an asymptomatic car-
ulinemia, amyloidosis, or other lymphoprolif- rier with no hematologic manifestations, as
erative disorders. MGUS is a preneoplastic there are four α globin genes and two will still
condition. There is no accepted treatment for function properly. Results of electrophoresis
this disorder because one cannot predict which should rule out α-thalassemia minor.
patients will progress to develop multiple my-
Answer C is incorrect. β-Thalassemia minor
eloma.
(the heterozygous defect) would show de-
Answer A is incorrect. Bisphosphonates such creased but not absent HbA1. This is a less se-
as alendronate and risedronate are used to treat vere form of the disease.

HIGH-YIELD SYSTEMS
multiple myeloma, which is known to cause
Answer D is incorrect. Glucose 6-phosphate
bone destruction due to increased osteoclast
dehydrogenase deficiency would not present
activity. Bisphosphonates have been shown to
with abnormal hemoglobin electrophoresis.
decrease pain and fractures in multiple my-
eloma by reducing the number and activity of Answer E is incorrect. HbH is less severe than
osteoclasts. Hb Barts and is associated with three-gene de-
letion of α globin. The abnormal hemoglobin
Answer B is incorrect. Anticoagulation is not
molecule (HbH) contains four β chains. The
the recommended treatment for MGUS.
β-thalassemias are more prevalent in Mediter-
Answer C is incorrect. High-dose steroids are ranean populations and the α-thalassemias are
not a current treatment modality for MGUS. more prevalent in Asian and African popula-
tions.
Answer E is incorrect. Vinca alkaloids such as
vincristine and vinblastine are microtubule in- Answer F is incorrect. Hb Barts is the most se-
hibitors used in the treatment of some cancers. vere of the genotypes involving deletion of α

Hematology-Oncology
However, vinca alkaloids are not currently the globin genes; it is associated with complete ab-
recommended treatment for MGUS. sence of all α-globin chains. This results in the
absence of all hemoglobins that require this
48. The correct answer is B. Thalassemias are ge- chain, and in the formation of tetramers of the
netic diseases involving decreased synthesis or γ chain (normally a component of HbF). This
complete absence of either the α-globin chain condition leads to hydrops fetalis and intrauter-
or the β-globin chain. This patient has classic ine fetal death.
symptoms of thalassemia: hemolytic anemia,
hepatosplenomegaly, and “chipmunk facies,” 49. The correct answer is E. This patient had an
cased by extramedullary hematopoeisis in the acoustic schwannoma removed from the cere-
bones of the face. The requirement for blood bellopontine angle. Schwann cell tumors are
transfusions since birth should raise the suspi- the third most common primary intracranial
cion for β-thalassemia major in this patient, tumor, are often localized to cranial nerve VIII
but the hemoglobin electrophoresis results (acoustic neuroma), and are commonly seen at
alone can be used to arrive at this conclusion. the cerebellopontine angle. The most com-
This patient shows increased HbF (α-2 γ-2) mon signs and symptoms of schwannomas in-
and HbA2 (α-2 δ-2); thus, synthesis of the α clude hearing loss, tinnitus, vertigo, hydro-
chain is intact. Absence of HbA1 (α-2 β-2) sup- cephalus, and increased intracranial pressure.
ports an absence of β-chain synthesis and, Most are benign, slow-growing tumors that can
therefore, a diagnosis of β-thalassemia major. be resected. Histologically, two patterns are
Death in these individuals is often caused by found: (1) Antoni A, or spindle cells palisading
cardiac failure secondary to hemochromatosis. and forming a whirl appearance; and (2) An-
 278 Section II: Organ Systems • Answers

toni B, or loosely arranged tissue after degener- with the most common hyperplasias/tumors as-
ation in the tumor. The histopathologic de- sociated with multiple endocrine neoplasia
scription of this patient’s tumor is consistent (MEN) type IIA, also known as Sipple’s syn-
with Antoni A. drome. MEN IIA is associated with parathyroid
hyperplasia or tumor leading to hypercalcemia,
Answer A is incorrect. Medulloblastomas are
medullary carcinoma of the thyroid, and pheo-
highly malignant radiosensitive tumors that are
chromocytoma, which commonly causes ele-
typically found in the posterior fossa. These tu-
vated plasma catecholamine levels. The MEN
mors are of neuroectodermal origin, and his-
syndromes follow an autosomal dominant pat-
topathologic examination shows a rosette or
tern of inheritance and both MEN IIA and
perivascular pseudorosette pattern. Peak inci-
MEN IIB (MEN III) are linked to distinct mu-
dence occurs in childhood.
tations in the ret oncogene.
HIGH-YIELD SYSTEMS

Answer B is incorrect. Meningiomas are slow-

Answer A is incorrect. The erb-B2 oncogene is
growing tumors that most often occur in the
associated with breast, ovarian, and gastric car-
hemispheric convexities and parasagittal re-
cinomas.
gions. Rarely they can appear in the cerebello-
pontine angle. However, the histology would Answer B is incorrect. Mutations in the MEN
classically show psammoma bodies, or areas of I gene are found in patients with multiple en-
calcification. docrine neoplasia I, also known as Wermer’s
syndrome, which is characterized by hyperpla-
Answer C is incorrect. Neurofibromas are tu-
sia or tumor of the “3 P’s”: Pituitary, Parathy-
mors of peripheral origin. This patient’s tumor
roid, and Pancreas.
is intra-axial, as shown on the CT scan. Histo-
logically, these cells appear as loosely arranged Answer C is incorrect. The ras oncogene mu-
spindle cells with intervening collagen. tation is associated with carcinoma of the co-
lon.
Answer D is incorrect. Oligodendrogliomas
are relatively uncommon, slow-growing tumors Answer D is incorrect. The Rb gene is a tumor
Hematology-Oncology

that occur most often in the frontal lobes. The suppressor gene located on chromosome 13q.
tumor comprises homogeneous sheets of cells Mutation of the gene is associated with retino-
with uniformly rounded nuclei and an associ- blastoma and osteosarcoma.
ated network of finely branching blood vessels.

50. The correct answer is E. The characteristics

described in the question are found in patients