HEMATOLOGIC DISORDERS

Medical-Surgical Nursing
 HEMATOLOGIC DISORDERS

• Section objective
– Anatomic and physiologic overview
– Assessment of hematologic system
– Disorders of the red blood cells
– Leukemias
– The lymphomas
– Bleeding disorders
– Therapies for blood disorders
– Summary
– Review questions
 Anatomy and physiology overview of blood

• Hematologic system consists of

• Blood
– Where produced i.e bone marrow and reticuloendothelial
system
– Blood-specialized organ
– Differs from other organs-exists in a fluid state.
– Composed of plasma and cells.
• Plasma-
– Fluid portion of blood; contains various proteins, such as
albumin, globulin, fibrinogen, and other factors necessary for
clotting, as well as electrolytes, waste products, and nutrients.
– About 55% of blood volume is plasma.
 BLOOD

• Body fluid circulating through the heart, arteries, capillaries,

and veins, consists of plasma and cellular components.
• Average male adult has about 5.5 L of blood;
• Average female 4.5 L.
• Plasma, fluid portion, accounts for 55% of the blood
volume, composed of;
– 92% water,
– 7% protein,
– 1% inorganic salts; nonprotein organic substances such
as urea; dissolved gases; hormones; and enzymes.
 CONT

• Plasma proteins;
– Albumin,
– Fibrinogen,
– Globulins.
• Cellular components
– Erythrocytes (red blood cells [RBCs]),
– Leukocytes and lymphocytes (white blood cells [WBCs]),
– Platelets.
• Derived from pluripotent stem cells in bone marrow, process-
hematopoiesis.
• Under normal conditions, only mature cells are found in circulating
blood.
• Cellular components of blood account for 45% of the blood volume.
 Functions of blood

• Regulating body temperature by transferring heat from deep within

the body to small vessels near the skin
• Providing cell nutrition by carrying nutrients from the
gastrointestinal (GI) tract to the tissues and removing waste
products by transporting them to the lungs, kidneys, liver, and skin
for excretion
• Defending the body against foreign antigens by transporting
leukocytes and antibodies to the sites of infection, injury, and
inflammation
• Transporting hormones from endocrine glands to various parts of
the body
• Maintaining acid-base balance
• Carrying oxygen to tissues and removing carbon dioxide
 Cellular component of blood

• Three primary cell types;

• Erythrocytes (RBCs)
– Enucleated, biconcave disc.
– Approximately 5 million erythrocytes per cubic millimeter of
blood.
– Cell contents -primarily of hemoglobin, essential for oxygen
transport.
– Whole blood contains 14 to 15 g of hemoglobin per 100 mL of
blood.
– Circulate about 115 to 130 days before elimination by
reticuloendothelial system, primarily in spleen and liver
 Leukocytes (WBC)
• Approximately 5,000 to 10,000 leukocytes (WBCs) per cubic millimeter
of blood.
• Classified ;
– Granulocytes leukocytes;
• Account for about 70% of all WBCs;
• Have abundant granules in cytoplasm; include neutrophils,
basophils, and eosinophils.
– Mononuclear leukocytes
• Have single-lobed nucleus and granule-free cytoplasm; include
monocytes and lymphocytes.
• Platelets (Thrombocytes)
– Approximately 150,000 to 450,000 platelets per cubic millimeter of
blood.
– Small particles without nuclei arise as a result of budding from giant
cells (megakaryocytes) in bone marrow.
– Primary function is to control bleeding through hemostasis
 Assessment of hematologic System

• History
• Subjective Data
– Disruption of the hematologic, immune, or coagulation
system,
– Vague complaints of fatigue,
– Frequent infections,
– Swollen glands, and bleeding tendencies.
 Review of Systems

• Skin and mucous membranes: Any bruises, infections, drainage, or

bleeding from wound sites?
• Neurologic: dizziness, tingling or numbness (paresthesia), headache,
forgetfulness or confusion, difficulty walking (disturbance in gait),
tiredness (fatigue), weakness?
• Respiratory: Experiencing shortness of breath, especially on exertion?
• Cardiovascular: Chest pain or feelings of funny heart beats in your
chest (palpitations)?
• GI: Any bleeding from gums, abdominal pain, black stools, or blood-
streaked vomit (emesis)? mouth sores, rectal pain, or diarrhea?
• GU: Menstrual flow. How often change pads? blood in urine or
discomfort on urination?
 History Questions

• Present medications? over-the-counter (OTC) medications,

vitamins, herbals, or nutritional supplements?
• Medical problems in the past?
• Surgery? partial or total gastrectomy, splenic injury or
splenectomy, tendency to bleed (e.g., with dental procedures),
infectious diseases, human immunodeficiency virus (HIV)
infection, cancer.
• Occupation? exposure to substances, such as benzene,
pesticides, and ionizing radiation.
• Family history of hematologic or malignant disorder?
• Social history and lifestyle, illicit drugs or alcohol? sexual
activity?
 Physical Examination

• May produce abnormal findings in various body systems due

to ;
– Anemia,
– Uncontrolled bleeding or clotting,
– Altered immune function that leads to infection in patients
with hematologic disorders.
 Examination Findings

• Anemia
– Tachycardia;
– Dyspnea;
– Shiny smooth tongue;
– Ataxia;
– Pallor of conjunctivae, nail beds, lips, and oral mucosa
• Altered clotting.
– Decreased blood pressure (BP),
– Altered level of consciousness (LOC),
– Hematuria, tarry stools,
– Petechiae, bleeding sites .
• Infection.
– Fever; tachycardia;
– Abnormal breath sounds;
– Delirium; oral lesions; erythema, swelling, tenderness, and drainage of the
skin
 Diagnostic Tests

• Laboratory Studies
– Complete blood count (CBC),
– Blood smear, and iron profile.
– Blood samples obtained by venipuncture.
• Complete Blood Count
• Absolute numbers or percentages of erythrocytes, leukocytes,
platelets, hemoglobin, and hematocrit in blood sample.
– Erythrocyte (RBC) indices
• Done to provide information on size, hemoglobin
concentration, and hemoglobin weight of an average RBC;
aids in diagnosis and classification of anemias.
 CONT

• Leukocyte (WBC) differential

– Done to determine the percentage of each type of
granulocyte (neutrophils, eosinophils, and basophils) and
non-granulocytes (lymphocytes and monocytes).
– Evaluate infection or potential for infection and identify
various types of leukemia.
 Blood Smear

• Blood specimen prepared for microscopic viewing using

appropriate stains,
• Allow visual analysis of numbers and characteristics of cells;
• Identify abnormal cells of certain anemias, leukemias, and
other disorders that affect the blood stream.
 Iron Profile

• Test completed on blood sample that generally includes levels

of serum ferritin, iron, total iron-binding capacity, folate,
vitamin B2,
• used to determine type and severity of anemia.
 Nursing and Patient Care Considerations

• Recent administration;
– Chloramphenicol,
– Hormonal contraceptives,
– Iron supplements,
– Corticotropin (ACTH) may affect results of serum iron
and iron-binding capacity. No patient preparation needed.
Bone Marrow Aspiration
• From the iliac crest or sternum to obtain specimen to
examine microscopically and to perform a biopsy.
 CONT

• Purposes include
– Diagnosis of hematologic disorders;
– Monitoring of course of illness and response to treatment;
– Diagnosis of other disorders, such as primary and metastatic
tumors, infectious diseases, and certain granulomas;
– Isolation of bacteria and other pathogens by culture.
• Nursing and Patient Care Considerations
– Give medication for pain and anxiety before or after the
procedure.
– A bone marrow aspiration with biopsy is more painful and
may require the use of conscious sedation with appropriate
monitoring.
– Watch for bleeding and hematoma formation after procedure.
 Lymph Node Biopsy

• Surgical excision or needle aspiration usually of a superficial

lymph node in the cervical, supraclavicular, axillary, or
inguinal region.
• Performed to determine the cause of lymph node enlargement,
• Distinguish between benign and malignant lymph node tumors
• Stage metastatic carcinoma.
 Disorders of The Red Blood Cells

• Include anemia and polycythemias.

Anemia
• Lack of sufficient circulating hemoglobin to deliver oxygen to
tissues.
• Could be due to inadequate production of RBCs or abnormal
blood loss.
• Common types in adults are;
– Iron deficiency anemia,
– Pernicious anemia,
– Folic acid deficiency
– Aplastic anemia,
 Etiologic categories:

• Loss of RBCs—occurs with bleeding, potentially from any

major source, e.g. GIT, uterus, nose, or a wound
• Decreased production of RBCs—caused by a deficiency in
cofactors (including folic acid, vitamin B12, andiron) required
for erythropoiesis; RBC production may also be reduced if the
bone marrow is suppressed (e.g., by tumor, medications, toxins)
or is inadequately stimulated because of a lack of erythropoietin
(as occurs in chronic renal disease).
• Increased destruction of RBCs—may occur because of an
overactive RES (including hypersplenism) or because the bone
marrow produces abnormal RBCs that are then destroyed by
the RES (e.g., sickle cell anemia).
 Iron Deficiency Anemia (Microcytic,
Hypochromic)

• Characterized by reduced total body iron content is decreased below

the normal level.
• This affects affecting hemoglobin synthesis and RBCs appear pale
and small.
• Pathophysiology and Etiology
• Common cause is chronic blood loss (GI bleeding, excessive
menstrual bleeding, hookworm infestation),
• May also be caused by insufficient intake of iron (weight loss,
inadequate diet),
• Iron mal-absorption (small bowel disease, gastroenterostomy), or
increased requirements (pregnancy, periods of rapid growth).
• Decreased hemoglobin may result in insufficient oxygen delivery to
body tissues.
 CONT

• Iron deficiency anemia, most common type of anemia, found

in 10% to 30% of all adults in the world.
• It is a major health problem in developing countries.
• Symptoms generally develop when hemoglobin has fallen to
less than 11 g/100 ml.

• Clinical Manifestations of Iron Deficiency Anemia

– Tinnitus, dizziness headache, fatigue.
– Palpitations, dyspnea on exertion, pallor of skin and
mucous membranes
– Spoon-shaped fingernails (Koilonychia )
– Smooth, sore tongue; lesions at corners of mouth (cheilosis)
– Craving to eat unusual substances (Pica)
 Medical Management of Iron Deficiency
Anemia

• Correction of chronic blood loss; Transfusion therapy with packed

RBCs, if necessary
• Oral or parenteral iron therapy.
– Oral ferrous sulfate preferred and least expensive; treatment
continues until hemoglobin level is normalized and iron stores
replaced (up to 6 months).
– Parenteral therapy may rarely be used when patient cannot tolerate
or is noncompliant with oral therapy. May use iron dextran
(Imferon)
• Diet: high in iron, fiber, and protein with increased fluids; avoidance
of teas and coffee, which reduce absorption of iron
• Vitamins: pyridoxine (vitamin B6), ascorbic acid (vitamin C)
 Nursing Management.

• Nursing Assessment
– Enquire about history of symptoms, dietary intake and possible sources
of blood loss.
– Observe for signs of GI or other bleeding, tachycardia, pallor and
dysnoea.
• Nursing Diagnoses
– Imbalanced Nutrition: less than body requirements related to
inadequate dietary iron intake.
– Activity Intolerance related to decreased oxygen-carrying capacity of
the blood
– Ineffective tissue perfusion related to decreased oxygen-carrying
capacity of the blood
• Interventions and Rationales
– Monitor intake and output to detect fluid imbalances.
– Monitor cardiovascular and respiratory status to detect decreased
activity intolerance and dyspnea on exertion.
 CONT

• Monitor and record vital signs to determine activity intolerance.

• Monitor stool, urine, and vomitus for occult blood to identify the
cause of anemia.
• Administer oxygen, as necessary, to treat hypoxemia caused by
reduced Hb.
• Provide a diet high in iron to replace iron stores in the body.
• Administer medications, as prescribed, to replace iron stores in the
body.
• Administer iron injection deep into muscle using Z-track technique
to avoid subcutaneous irritation and discoloration from leaking drug.
• Administer liquid preparations with a straw to prevent staining of the
teeth.
 CONT

• Encourage fluids to avoid dehydration.

• Provide rest periods to avoid fatigue and reduce oxygen
demands
• Provide mouth, skin, and foot care because the tongue or lips
may be dry or inflamed and the nails may be brittle.
• Protect the patient from falls caused by weakness and fatigue.
Falls may result in breeding and bruising.
• Keep the patient warm to enhance comfort.
 Diagnostic Findings of Iron Deficiency
Anemia

• Hematology shows decreased Hb, HCT, iron, ferritin, reticulocytes,

red cell indices, transferrin, and saturation; absent hemosiderin; and
increased iron-binding capacity.
• Peripheral bloods smear reveals microcytic and hypochromic RBCs.
Teaching topics for a patient with Iron Deficiency Anemia
– Eating foods rich in iron and vitamin C
– Recognizing signs and symptoms of bleeding
– Monitoring stools for occult blood
– Recognizing stool color changes caused by oral iron
supplementation (stools will be black)
– Refraining from using hot pads and hot water bottles
– Preventing constipation
 Megaloblastic Anemias

• Caused by deficiencies of vitamin B12 or folic acid,

• Identical bone marrow and peripheral blood changes occur, because
both vitamins are essential for normal DNA synthesis.
• In either anemia, the RBCs that are produced are abnormally large and
are called megaloblastic RBCs.
• Other cells derived from the myeloid stem cell (nonlymphoid WBCs,
platelets) are also abnormal

Megaloblastic Anemia: Pernicious

• A megaloblast is a large, nucleated erythrocyte with delayed and
abnormal nuclear maturation.
• Pernicious anemia is a type of megaloblastic anemia associated with
vitamin B2 deficiency.
 Pathophysiology and Etiology

• Vitamin B12 is necessary for normal deoxyribonucleic acid

synthesis in maturing RBCs.
• Pernicious anemia demonstrates familial incidence related to
autoimmune gastric mucosal atrophy.
• Normal gastric mucosa secretes a substance called intrinsic
factor, necessary for absorption of vitamin B12 in ileum.
• If a defect exists in gastric mucosa, or after gastrectomy or
small bowel disease, intrinsic factor may not be secreted and
orally ingested B12 not absorbed. and RBC production is
eventually diminished.
 CONT

• Adequate vitamin B12 and intrinsic factor are present, a

deficiency may occur if disease involving the ileum or
pancreas impairs absorption.
• Pernicious anemia, which tends to run in families, is primarily
a disorder of adults, particularly the elderly.
• Some drugs interfere with B12 absorption, notably ascorbic
acid, cholestyramine, colchicine, neomycin, cimetidine, and
hormonal contraceptives.
Clinical Manifestations for Pernicious Anemia

• Signs of anemia (palpitations, pallor, fatigue, dyspnea on

exertion)
• Underlying GIT(sore mouth, glossitis, anorexia, nausea,
vomiting, gastrectomy, small bowel malfunction)
• Neuropathies (paresthesia, gait disturbance, bladder and bowel
dysfunction)
Diagnostic Evaluation for Pernicious Anemia
• Complete Blood Count (CBC) Blood tests show decreased
hemoglobin and hematocrit;
• Marked variation in size and shape of RBCs (megaloblasts)
 CONT

• Reduced B12 levels normal levels of folic acid.

• Gastric analysis shows reduced volume and acidity of gastric
juice.
• Schilling test for absorption of vitamin B12 in which
radioactive B12 is given orally and 24-hour urine is collected
for analysis shows decreased amounts.
 Nursing Management for Pernicious Anemia

• Nursing assessment
– Assess for pallor, tachycardia, dyspnea on exertion, exercise intolerance
to determine patient's response to anemia.
– Assess for paresthesia, gait disturbances, changes in bladder or bowel
function, altered thought processes indicating neurologic involvement.
– Obtain history of gastric surgery or GI disease
• Nursing Diagnoses
– Disturbed thought processes related to neurologic dysfunction in
absence of vitamin B12
– Impaired Sensory Perception related to neurologic dysfunction in
absence of vitamin B12
• Nursing Interventions
– Improving Thought Processes by administering vitamin B12 as
prescribed and nurse patient in a quiet, supportive environment.
– Minimizing the Effects of Paresthesia
– Educate patient and health maintenance
 CONT

• Advise patient that monthly vitamin B12 administration

throughout life time.
• Instruct patient to biannual for hematologic studies and GI
evaluation
• Examine patient periodically for gastric cancer and thyroid
dysfunction usually associated with pernicious anemia
Take Note
• Patients with pernicious anemia have higher incidence of
gastric cancer and thyroid dysfunction; periodic stool
examinations for occult blood, gastric cytology, and thyroid
function tests are done
 Medical Management.

• Parenteral hydroxocobalamin or cyanocobalamin (B12) is given

monthly intramuscularly.
• Vitamin B12 deficiency is treated by vitamin B12 replacement. Usually
at a dose of 1000 μg.
• Vegetarians can prevent or treat deficiency with oral supplements
through vitamins or fortified soy milk.
• The reticulocyte count rises within 1 week, and in several weeks the
blood counts are all normal.
• The tongue improves in several days.
• However, the neurologic manifestations require more time for recovery;
if there is severe neuropathy, the patient may never recover fully.
• To prevent recurrence of pernicious anemia, vitamin B12 therapy must
be continued for life.
 Megaloblastic Anemia: Folic Acid Deficiency

• Chronic megaloblastic anemia caused by folic acid deficiency.

Pathophysiology and Etiology of Folic Acid Deficiency
• Dietary deficiency, malnutrition, overcooking foods and
alcoholism cause folate deficiency
• Impaired absorption in jejunum in small bowel disease
• Increased requirements e.g. in chronic hemolytic anemia and
pregnancy
• Use of antimetabolite drugs e.g. methotrexate antagonizes folic
acid.
• Other drugs include phenytoin, broad spectrum antibiotics,
sulfamethoxazole, alcohol, and hormonal contraceptives).
 Clinical Manifestations of Folic Acid
Deficiency

– Signs of anemia: fatigue, weakness, pallor, dizziness, headache,

tachycardia.
– Signs of folic acid deficiency: sore tongue, cracked lips.
Diagnostic Evaluation of Folic Acid Deficiency
– Vitamin B12 and folic acid level€”folic acid will be decreased.
– CBC will show decreased RBC, hemoglobin, and hematocrit
with increased mean corpuscular volume and mean corpuscular
hemoglobin concentration.
Medical Management of Folic Acid Deficiency
• Oral folic acid supplement on daily basis.
• Folate deficiency is treated by increasing the amount of folic acid
in the diet and administering 1 mg of folic acid daily.
• Folic acid is administered intramuscularly only for people with
malabsorption problems.
 CONT

• With the exception of the vitamins administered during

pregnancy, most proprietary vitamin preparations do not contain
folic acid, so it must be administered as a separate tablet.
• After the hemoglobin level returns to normal, the folic acid
replacement can be stopped.
• However, patients with alcoholism should continue receiving
folic acid as long as they continue alcohol consumption.

• Nursing management of patient with folic acid deficiency

• Nursing Assessment
– Obtain information about patient’s nutritional history.
– Assess patient for dyspnea, and development of chest pain or
shortness which are indicators for worsening of condition
 Nursing Diagnosis

• Imbalanced nutrition: less than body requirements related to

inadequate intake of folic acid
• Nursing management of Folic Acid Deficiency
• To improve Folic Acid Intake you;
– Assess diet for inclusion of foods rich in folic acid: beef liver,
peanut butter, red beans, oatmeal, broccoli, and asparagus.
– Arrange nutritionist referral as appropriate.
– Assist alcoholic patient to obtain counseling and additional
medical care as needed.
• Encourage pregnant patient to maintain prenatal care and to take
folic acid supplement.
• Provide alcoholic patient with information about treatment
programs and Alcoholics Anonymous meetings in the community.
• Teach patient to select balanced diet that includes green vegetables
(asparagus, broccoli, spinach), yeast, liver and other organ meats,
some fresh fruits; avoid overcooking vegetables.
 Aplastic Anemia

• Disorder characterized by pancytopenia (depleted RBCs,

WBCs, and platelets) due to bone marrow hypoplasia.
• Pathophysiology and Etiology
• Immune-mediated mechanism causing destruction of
hematopoietic stem cells.
• Cause is not known but may be associated with exposure to
chemical toxins;
• Ionizing radiation; viral infections and certain drugs for
example, chloramphenicol.
 Clinical Manifestations

• Signs of anemia (pallor, weakness, fatigue, exertional dyspnea,

palpitations).
• Signs of infection with neutropenia (fever, headache, malaise)
• Signs of thrombocytopenia (bleeding from gums, nose, purpura,
petechiae, ecchymoses).

Diagnostic Evaluation
• CBC, Peripheral blood smear show decreased RBC, WBC,
platelets (pancytopenia).
• Bone marrow aspiration and biopsy shows hypocellular or empty
cells with greatly reduced or absent hematopoiesis.
 Medical Management

• Removal of causative agent or toxin.

• Allogeneic bone marrow transplantation (BMT) treatment of
choice for patient with severe aplastic anemia.
• This treatment option provides long-term survival for 75% to
90% of patients, depending on the age of the patient, history of
prior blood transfusions, and source of marrow.
 CONT

• Immunosuppressive treatment with corticosteroids,

cyclosporine, cyclophosphamide, antithymocyte globulin or
antilymphocyte globulin as single treatments or in combinations.
• This treatment option provides long-term survival for 70% to
80% of patients.
• Androgens (oxymetholone or testosterone enanthate) may
stimulate bone marrow regeneration; significant toxicity
encountered.
• They may be used when other treatments have failed.
• Supportive treatment includes platelet and RBC transfusions,
antibiotics, and antifungals.
 Nursing Management

• Nursing Assessment
– Obtain thorough history about medications, past medical
history, occupation, hobbies.
– Monitor for signs of bleeding and infection.
• Nursing Diagnoses
– Risk for Infection related to granulocytopenia secondary to
bone marrow aplasia
– Risk for Injury related to bleeding
 Nursing interventions and Rational

• Main goal is to Minimizing Risk of Infection, Minimizing Risk of

Bleeding and Patient education and health maintenance
• Monitor respiratory status to detect hypoxemia caused by low Hb
levels.
• Monitor vital signs for signs of hemorrhage, infection, and activity
intolerance.
• Monitor and record intake and output and urine specific gravity to
determine fluid balance.
• Check stool, urine, and vomitus for occult blood loss caused by
reduced platelet levels.
• Monitor for infection, bleeding, and bruising caused by reduced
levels of WBCs and platelets.
• Encourage fluids and maintain I.V. fluids to replace fluids lost by
fever and bleeding.
 CONT

• Administer oxygen to improve tissue oxygenation because low

Hb levels reduce the oxygen-carrying capacity of the blood.
• Assist with turning, coughing, and deep breathing to mobilize and
remove secretions.
• Monitor transfusion therapy, as prescribed, to replace low blood
components.
• Maintain the patient’s diet to promote RBC production and fight
infection.
• Encourage verbalization of concerns and fears to allay the
patient’s anxiety.
• Alternate rest periods with activity to conserve energy and reduce
weakness caused by anemia.
 CONT

• Provide cooling blankets and tepid sponge baths for fever to promote
comfort and reduce metabolic demands.
• Maintain protective precautions to prevent infection and hemorrhage.
• Provide mouth care before and after meals to enhance the taste of
meals.
• Provide skin care to prevent skin breakdown due to bed rest,
dehydration, and fever.
• Protect the patient from falls to reduce the risk of hemorrhage.
• Avoid giving the patient I.M. injections to reduce the risk of
hemorrhage.
• Avoid using hard toothbrushes and straight razors on the patient to
reduce the risk of hemorrhage.
 Teaching topics

• Recognizing the early signs and symptoms of bleeding and

infection
• Avoiding contact sports
• Wearing a medical identification bracelet
• Monitoring stool for occult blood
• Using an electric razor to avoid bleeding
• Refraining from taking aspirin or nonsteroidal anti-
inflammatory drugs (NSAIDs)
 Sickle cell anemia

• Sickle cell anemia is a hereditary hematologic disease that

causes impaired circulation,
• Chronic ill health, and premature death. Although it’s most
common in tropical Africa and in people of African descent, it
also occurs in people from Puerto Rico, Turkey, India, the
Middle East, and the Mediterranean.
 Pathophysiology

• Change in gene that encodes beta chain of Hb results in a

defect in hemoglobin S (Hobs) cell.
• When hypoxia (oxygen deficiency) occurs, the HbS in the
RBCs becomes insoluble.
• Consequently, the RBC containing (HbS) loses its round, very
pliable, biconcave disk shape and becomes deformed, rigid,
and sickle-shaped.
CONT
 CONT

• Cells become rigid and rough, forming an elongated sickle

shape and impairing circulation.
• Infection, stress, dehydration, and conditions that provoke
hypoxia:-strenuous exercise, high altitude, unpressurized
aircraft, cold, and vasoconstrictive drugs, may all provoke
periodic crises.
• Crises can occur indifferent forms, including painful crisis,
aplastic crisis, and acute sequestration crisis.
 Causes

• Genetic inheritance: results from homozygous inheritance of

an autosomal recessive gene that produces a defective Hb
molecule(HbS); heterozygous inheritance results in sickle cell
trait (people with this trait are carriers who can then pass the
gene to their offspring)
 Signs and Symptoms

• Aching bones
• Chronic fatigue
• Family history of the disease
• History of frequent infections
• Jaundice, pallor
• Joint pain and swelling
• Leg ulcers (especially on ankles)
• Severe localized and generalized pain
• Tachycardia
• Unexplained dyspnea or dyspnea on exertion
• Unexplained, painful erections (priapism)
 Sickle cell crisis (general symptoms)

• Hematuria
• Irritability
• Lethargy
• Pale lips, tongue, palms, and nail beds
• Severe pain
• Painful crisis (vaso-occlusive crisis, which appears periodically
after age 5)
– Dark urine, Low-grade fever
– Severe abdominal, thoracic, muscle, or bone pain
– Tissue anoxia and necrosis, caused by blood vessel obstruction
by tangled sickle cells
– Worsening of jaundice
Aplastic crisis (generally associated with viral
infection)

• Dyspnea
• Lethargy, sleepiness
• Pallor
• Possible coma
• Acute sequestration crisis (rare; occurs in infants ages 8 months to 2
years)
• Signs and symptoms of hypovolemic shock
– Lethargy
– Liver enlargement
– Pallor
– Worsened chronic jaundice
 Diagnostic Findings of sickle cell anemia

• Blood tests show

– Low RBC counts,
– Elevated WBC and platelet counts,
– Decreased ESR,
– Increased Serum iron levels,
– Decreased RBC survival, and reticulocytosis.
• Hb electrophoresis shows
– HbS.
– Hb levels may be low or normal.
• Stained blood smear shows sickle cells.
 Nursing Diagnoses of sickle cell anemia

• Impaired gas exchange

• Acute pain
• Ineffective tissue perfusion: Peripheral
 Medical Management of sickle cell anemia

Acute management
• Pain management:
– Opiates-Morphine, fentanyl, hydro morphine, Meperidine not
recommended(CNS toxicity)
• Fluid therapy
– IV fuids in severe painful crisis – 5% dextrose and normal saline: 1.2
times maintainance
• Treatment of infection:
– Broad spectrum antibiotics
– Acute chest syndrome S. pneumoniae, H. influenzae type b,
Mycoplasma pneumoniae, and Chlamydia pneumoniae (cefuroxime
and erythromycin)
– Osteomyelitis- salmonella and s. aureus
 CONT

• Transfusion therapy(therapeutic)
– Simple transfusion is used for single transfusions to restore
oxygen carrying capacity or blood volume
– Partial exchange transfusions are recommended for acute
emergencies and for chronic transfusion because of the
improved viscosity effects and reduced iron burden with
this approach
– Hb should not be raised much above 10 g/dL because of
increases in viscosity and the risk of vasoocclusive
episodes.
 Long term management of sickle cell anemia

• Infection control and prophylaxis

– Instructions regarding early recognition of infection. Fever
should be considered a medical emergency requiring
prompt medical attention and treatment with antibiotics.
– Immunization against Streptococcus pneumoniae,
Haemophilus influenzae type B, hepatitis B virus, and
influenza .
 CONT

• Prophylactic penicillin — Penicillin V should be given 125 mg

per oral twice a day within 3 months of birth until 2-3 yrs of
age then 250 mg twice per day until the age of five.
– After five years of age may elect to stop penicillin
prophylaxis.
– Evidence in favor of stopping penicillin prophylaxis is
sparse, given a life-long persistence of splenic dysfunction
starting in late childhood and continuing through
adulthood.
 CONT

• Routine treatment and evaluations of sickle cell anemia

• Folic acid is given orally in a dose of 1 mg/day
• Bone marrow transplant:
– Allogeneic Bone Marrow Transplant from matched sibling
donor cures 85% of children with sickle cell disease (SCD)
less than 16 years of age
– Indications for bone marrow transplant.
• Stroke
• Recurrent acute chest syndrome
• Recurrent vaso-occlusive episodes
 Nursing Interventions and Rationales

• Provide emotional support to allay the patient’s anxiety.

• Refer the patient for genetic counseling to decrease anxiety
and help the patient to understand the chances of passing the
disease to offspring.
• Refer the patient and his family to community support groups
to help the patient and his family cope with his illness.
 During a crisis

• Apply warm compresses to painful areas, and cover the patient

with a blanket. Cold compresses and temperature can
aggravate the condition.
• Administer an analgesic-antipyretic, such as aspirin or
acetaminophen, for pain relief. (Additional pain relief may be
necessary during an acute crisis.)
• Maintain bed rest to reduce workload on the heart and reduce
pain.
 CONT

• Monitor the administration of blood components(packed

RBCs), as ordered, for aplastic crisis caused by bone marrow
suppression.
• Administer oxygen to enhance oxygenation and reduce sickling.
• Encourage fluid intake to prevent dehydration, which can
precipitate a crisis.
• Maintain prescribed I.V. fluids to ensure fluid balance and renal
perfusion.
• Give antibiotics, as ordered, to treat infections and avoid
precipitating a crisis.
 Teaching topics for a patient with sickle cell
anemia

• Avoiding circulation restriction

• Receiving normal childhood immunizations
• Obtaining prompt treatment for infections
• Maintaining increased fluid intake to prevent
dehydration
• Preventing hypoxia, Avoiding known triggers
 Assignment

• Polycythemia,
• Congenital anemia and
• Hemolytic anemias due to intrinsic red blood cells defects.
 Leukemia

• Refer to malignant disorders of the blood and bone marrow characterized, by

accumulation of dysfunctional, immature cells that have lost their ability to
multiply.
• Leukemias are commonly classified according to the stem cell line involved,
either;
– Lymphoid
– Myeloid.
• Also classified as either
– Acute
– Chronic,
• Based on the time it takes for symptoms to evolve and phase of cell
development that is halted (i.e., with few WBCs differentiating beyond that
phase).
• Acute lymphocytic leukemia (ALL), lymphocytes are the predominant cells.
• Acute myelogenous leukemia (AML)/acute non-lymphocytic leukemia.-
dominance of monocytes or granulocytes
 Etiology and pathophysiology

• Incidence highest in children age 2-6 years.; declines until age

35 at which point there is a steady increase
• Etiology unknown, exposure to certain toxic substances such
as radiation seems to increase the incidence.
• In general uncontrolled proliferation of white blood cells and
is classified according to the type of white blood cell affected
shown as follow;
• edit
 CONT

• Acute myeloid leukemia (AML): occur throughout life cycle.

• All age groups are affected; the incidence rises with age, with a
peak incidence at age 60 years.
• AML is most common non-lymphocytic leukemia.
• Prognosis is poor without chemotherapy; leukocytes are
premature and abnormal.
• Chronic myeloid leukemia (CML): occurs after the second
decade;
• Prognosis is poor; results from abnormal production of
granulotic cells.
• Acute lymphocytic leukemia (ALL): primarily occur in children
 CONT

• Onset of symptoms is abrupt, often occurring

within a few weeks.
• WBC development is halted at the blast phase,
so that most WBCs are undifferentiated or are
blasts.
• Acute leukemia progresses very rapidly; death
occurs within weeks to months without
aggressive treatment.
 CONT

• Chronic lymphocytic leukemia (CLL): occurs most

commonly in 50-70 years old.
• Life expectancy is 4-5 years; results from increased production
of leukocytes and lymphocytes and proliferation of cells
within the bone marrow, spleen and liver.
• Symptoms evolve over a period of months to years, and the
majority of WBCs produced are mature.
• Chronic leukemia progresses more slowly; the disease
trajectory can extend for years
Clinical Manifestations
 Diagnostic Evaluation

• Full blood counts (FBC) film, differential WBC count,

including platelets,
– Maybe nucleated RBC, myelocytes, as well as blasts.
– May simply be pancytopenic without blasts (aleukaemic
leukaemia) – commoner in ALL.
• Bone marrow aspirate for:
– Routine morphology and cytochemistry.
• Where available:
– Chromosome studies (especially AML and CGL in
transformation).
 CONT

– Immunological markers (e.g. cALL antigen, T and B markers, Ia

marker).
– Enzyme assays (e.g. TdT in ALL; lyzozyme in AML).
– In vitro culture.
– Serum lyzozyme level (AML) especially monocytic).
• Bone marrow trephine may give additional information on degree of
infiltration. May show secondary fibrosis.
Once diagnosis established various baseline investigations relevant to
future management should be initiated.
• Haematological Tests
– Blood group and antibody screen.
– HLA type (necessary for transplantation, blood products; often not
possible until remission because of low lymphocyte count).
– Coagulation screen to exclude DIC.
Tests to be considered even though may not be
performed.

• Biochemical Tests
– Renal function tests.
– Uric acid LDH.
– Liver function test.
– Serum calcium (magnesium if available).
• Microbiological Tests
– Superficial surveillance swabs – include mouth, skin, nose,
perineum.
– Urine and stool culture.
– Blood cultures (and any other clinically indicated) if febrile.
– Baseline viral titres (especially HSV and CMV).
– Hepatitis surface antigen.
 Radiological Tests

• Chest X-ray.
• Others where clinically indicated.
Principles of management
• Two main categories:
– Supportive treatment.
– Definitive anti-leukaemic treatment.
 Treatment accompaniment / Supportive

• Maintain Hb>10g/dl by transfusion of packed cells.

• Platelet transfusions are indicated when:
– There is clinical evidence of bleeding e.g. purpura.
– When transfusion with stored blood may dilute an already
low platelet count.
– Prophylactic platelet transfusions are often used when the
count fall below 20 x 109/L. Such use depends on local
availability and policy.
 CONT

• Prevent uric acid nephropathy and gout due to rapid cell lysis:
– Start allopurinol 300 daily preferably at least 24 hours before
chemotherapy.
– Ensure good fluid input and output with careful records of balance.
• Check electrolytes daily or more frequently during initial stages of
treatment as cell lysis may cause hyperkalaemia.
• Combinations of chemotherapy, antibiotics and infection lead to other
metabolic problems. Keep a check on:
– Renal and liver function.
– K+, Ca++, Mg++ levels.
– Albumin ( may reflect poor nutrition)
 CONT

• Coagulation screen (PT, APPT, TT):

– Liver infiltration, certain drugs e.g. asparaginase may cause
coagulation defects.
– DIC is common in promyelocytic leukaemia and may occur in
any AL especially after cell lysis.
– Heparin in low dose may be useful but must be very carefully
monitored.
• Arrange insertion of venous line access or Hickman catheter if
possible.
• Maintain the patient’s morale.
– Careful discussion and explanation of disease and treatment are
extremely important.
– Be honest and optimistic as far as reasonably possible.
– Use anti-emetics, analgesia and psychotropic drugs where
indicated.
 CONT

• Definitive anti-leukaemic treatment. Can be summarized as;

– Chemotherapy
– Transfusions of whole blood or blood fractions
– Analgesics
– Bone marrow transplant
– Radiation of areas of lymphocytic infiltration.
 Nursing Management of leukemia

• Nursing Assessment
– Take nursing history, focusing related symptoms
– Enquire about difficulty in swallowing, coughing, rectal pain.
– Examine patient for enlarged lymph nodes, liver and spleen,
evidence of bleeding, skin lesions and abnormal breath
sound.
– Look for evidence of infection in the mouth, tongue, and
throat, skin
– Baseline vital signs for; observe for signs of anemia,
thrombocytopenia, and neutropenia.
 Nursing Diagnoses

• Acute or chronic pain related to leukocytic infiltration of

systemic tissues.
• Fatigue related to anemia
• Risk for Infection related to granulocytopenia and treatment,
ineffective immune system
• Risk for Injury related to bleeding secondary to bone marrow
failure and thrombocytopenia
 Nursing Interventions and Rationale for a
patient with leukemia

• Monitor and record vital signs to promptly detect deterioration in

patient’s condition.
• Monitor intake, output, and daily weight because body weight may
decrease as a result of fluid loss.
• Monitor for bleeding. Regular assessment may help anticipate or
alleviate problems.
• Place the patient with epistaxis in an upright position leaning
slightly forward to reduce vascular pressure and prevent aspiration.
• Monitor for infection. Damage to bone marrow may suppress
WBC formation.
• Promptly report temperature over 101° F (38.3° C) and decreased
WBC counts so that antibiotic therapy may be initiated.
 CONT

• Monitor oxygen therapy. Oxygen therapy increases alveolar

oxygen concentration and enhances arterial blood oxygenation
• Encourage fluids to maintain adequate hydration.
• Maintain I.V. fluids to replace fluid loss.
• Encourage turning every 2 hours to prevent venous stasis and
skin breakdown.
• Encourage coughing and deep breathing to help remove
secretions and prevent pulmonary complications.
• Place the patient in semi-Fowler’s position when in bed to
promote chest expansion and ventilation of basilar lung fields.
 CONT

• Maintain the patient’s diet to provide necessary nutrition.

• Monitor Total Parenteral Nutrition, if needed, to provide the patient
with electrolytes, amino acids, and other nutrients tailored to his
needs.
• Monitor transfusion therapy for adverse reactions. Transfusion
reactions may occur during blood administration and may further
compromise the patient’s condition.
• Administer medications, as prescribed, to combat disease and
promote wellness.
• Provide gentle mouth and skin care to prevent oral mucous
membrane or skin breakdown.
• Encourage the patient to express his feelings about changes in his
body image and fear of dying to reduce anxiety.
• Avoid giving the patient I.M. injections and enemas and taking rectal
temperature to prevent bleeding and infection.
 Lymphomas

• Lymphomas are neoplasms of cells of lymphoid origin.

• Usually start in lymph nodes but can involve lymphoid tissue
in the spleen, the gastrointestinal tract (for example, the wall
of the stomach), the liver, or the bone marrow.
• Classified according to the degree of cell differentiation and
the origin of the predominant malignant cell.
• Broadly classified into two categories:
– Hodgkin’s disease and
– Non-Hodgkin’s lymphoma (NHL).
 Hodgkin’s lymphoma

• Malignant disorders of the reticuloendothelial system that

result in an accumulation of dysfunctional, immature
lymphoid-derived cells.
• Hodgkin's lymphoma originates in the lymphoid system .
• They are classified according to the predominant cell type and
by the degree of malignant cell maturity.
 Etiology and Pathophysiology

• Etiology is unknown, thought that the spread could be via

bloodstream from other sites.
• Higher incidence in male and young adults
• Proliferation of malignant cells (Reed-Sternberg cells) within
lymph nodes
• All tissues may eventually be involved but chiefly lymph
nodes, spleen, liver, tonsils and bone marrow
• Classification is by staging and the presences or absence of
symptoms
 Clinical Manifestations

• Painless enlargement of lymph nodes fever, chills, night

sweats, weight loss, purities.
• Symptoms of pulmonary involvement, superior vena cava
obstruction, hepatic or bone involvement.
Diagnostic Evaluation
– Tests are used to determine extent of disease involvement
before treatment and followed at regular intervals to assess
response to treatment.
– CBC determines abnormal cells.
– Lymph node biopsy determines type of lymphoma.
 CONT

• Bilateral bone marrow aspirate and biopsy determine whether bone

marrow is involved.
• Radiographic tests (for example, X-rays, computed tomography [CT]
scan, magnetic resonance imaging [MRI]) to detect deep nodal
involvement.
• Liver function tests, scan to determine hepatic involvement; liver
biopsy may be indicated if results abnormal.
• Lymphangiogram to detect size and location of deep nodes involved,
including abdominal nodes, which may not be readily seen via CT
scan.
• Surgical staging (laparotomy with splenectomy, liver biopsy, multiple
lymph node biopsies) in selected patients.
 Medical Management

• Choice of treatment depends on;

– Extent of disease,
– Histopathologic findings, and prognostic indicators.
• Hodgkin's lymphoma is more readily cured than other lymphomas,
with a 5-year survival of 80%.
• More than one treatment strategy is available, and combinations of
radiation and chemotherapy are commonly used.
• Hodgkin's lymphoma arising in presence of HIV requires specialized
treatment.
• Radiation therapy
– Treatment of choice for localized disease.
– Areas of body where lymph node chains are located can generally
tolerate high radiation doses.
– Vital organs are protected with lead shielding during radiation
 CONT

• Chemotherapy
– Three or four drugs may be given in intermittent or cyclical
courses with periods off treatment to allow recovery from
toxicities.
• Autologous or allogeneic bone marrow or stem cell
transplantation
• Surgical interventions include excision of masses to relieve
pressure on other organs.
• Potential side effects: nausea, skin rashes, dry mouth,
dysphagia, infections, and pancytopenia are managed.
 Nursing Management

Nursing Assessment
• Lymph nodes to determine enlargement
• Temperature for baseline data
• Liver and spleen to determine enlargement.
• Obtain focused health history, focusing on fatigue, fever, chills,
night sweats, swollen lymph nodes.
Nursing Diagnoses
• Activity intolerance related to effects of disease and side effects of
treatment
• Impaired Tissue Integrity related to high-dose radiation therapy
• Impaired Oral Mucous Membrane related to high-dose radiation
therapy
 Nursing Interventions for lymphoma

• To maintain tissue integrity you;

– Avoid rubbing, powders, deodorants, lotions, or ointments (unless
prescribed) or application of heat and cold to treated area.
– Encourage patient to keep treated area clean and dry, bathing area
gently with tepid water and mild soap.
– Encourage wearing loose-fitting clothes.
– Advise patient to protect skin from exposure to sun, chlorine,
temperature extremes.
• To preserving Oral and GI Tract Mucous Membranes you;
– Encourage frequent small meals, using bland and soft diet at mild
temperatures.
– Teach patient to avoid irritants, such as alcohol, tobacco, spices,
extreme food temperatures.
 CONT

• Administer or teach self-administration of pain medication or

antiemetic before eating or drinking, if needed.
• Encourage mouth care at least twice per day and after meals
using gentle flossing, soft toothbrush and mild mouth rinse.
• Assess for ulcers, plaques, or discharge that may be indicative
of superimposed infection.
• For diarrhea, switch to low-residue diet and administer
antidiarrheals as ordered.
 In Patient Education and Health
Maintenance;
• Teach patient about risk of infection
• Teach patient how to take medications as ordered, and instruct about
possible adverse effects and management.
• Explain to patient that radiation therapy may cause sterility; men
should be given opportunity for sperm banking before treatment;
women may develop ovarian failure and require hormone
replacement therapy.
• Reassure patient that fatigue will decrease after treatment is
completed; encourage frequent naps and rest periods.
• Patients need to be taught to minimize the risks for infection, to
recognize signs of possible infection, and to contact the health care
professional should such signs develop .
• However, as survival rates increase, the incidence of second
malignancies, also increases. Therefore, survivors should be
screened regularly for the development of second malignancies.
 Bleeding Disorders

• Normal hemostatic mechanisms can control bleeding from

vessels and prevent spontaneous bleeding.
• Bleeding vessel constricts and platelets aggregate at the site,
forming an unstable hemostatic plug.
• Circulating coagulation factors are activated on the surface of
these aggregated platelets, forming fibrin, which anchors the
platelet plug to the site of injury
CONT
 CONT

• Bleeding disorders refer to dysfunction in any phase of hemostasis.

• The disorder may be congenital or acquired.
• Bleeding disorders seen in adults include
– Thrombocytopenia,
– Idiopathic thrombocytopenic purpura (ITP),
– DIC, and
– Von Will brand’s disease.

Thrombocytopenia
• Thrombocytopenia is the most common cause of bleeding disorders
characterized by decreased platelet count (less than 100,000/mm3).
 CONT
• Thrombocytopenia (low platelet level) can result from various factors:
– Decreased production of platelets within the bone marrow,
– Increased destruction of platelets,
– Increased consumption of platelets.
• Clinical Manifestations
• Usually asymptomatic.
– When platelet count drops below 20,000/mm3:
• Petechiae occur spontaneously
• Ecchymoses occur at sites of minor trauma (venipuncture, pressure)
• Bleeding may occur from mucosal surfaces, nose, GI and GU tracts,
respiratory system, and within CNS
• Menorrhagia is common.
• Excessive bleeding may occur after procedures (dental extractions, minor
surgery, biopsies).
• Thrombotic complications (arterial and venous) and areas of skin necrosis
are associated with heparin-induced thrombocytopenia
 Diagnostic Evaluation

• Decreased platelet, hemoglobin, hematocrit count,

• Bleeding time, prothrombin time (PT), partial thromboplastin time
(PTT) is prolonged.
• Platelet aggregation test for heparin-dependent platelet antibodies is
positive.
Medical Management
– Treat underlying cause.
– Platelet transfusions.
– Steroids or I.V. immunoglobulins may be helpful in selected
patients.
– Heparin-induced thrombocytopenia: discontinue heparin, use
alternate anticoagulant therapy due to high risk of thromboses
(direct thrombin inhibitors, such as argatroban, avoid platelet
transfusions.
 Nursing Management.

• Nursing Assessment
– Obtain health history, focusing on prior illnesses and
episodes of bleeding, past surgical experiences, exposure to
toxins or ionizing radiation, family history of bleeding
– Obtain list of current and recent medications including over
the counter preparations, herbal and dietary supplements.
– Perform complete physical examination for signs of
bleeding.
• Nursing Diagnosis
– Risk for Injury related to bleeding due to thrombocytopenia
 Nursing Interventions

• Institute bleeding precautions.

– Avoid use of plain razor, hard toothbrush or floss, I.M.
injections, tourniquets, rectal procedures, and suppositories.
– Administer stool softeners as necessary to prevent constipation.
– Restrict activity and exercise when platelet count is less than
20,000/mm3 or when active bleeding occurs.
• Monitor pad count and amount of saturation during menses;
administer or teach self-administration of hormones to suppress
menstruation as prescribed.
• Administer blood products as ordered. Monitor for signs and
symptoms of allergic reactions, anaphylaxis, and volume overload.
• Evaluate urine, stool, and emesis for gross and occult blood
 CONT

• Perform patient education and health maintenance;

– Teach patient bleeding precautions (see Patient Education
Guidelines, page 945).
– Demonstrate the use of direct, steady pressure at bleeding
site if bleeding develops.
– Encourage routine follow-up for platelet counts.
Idiopathic thrombocytopenic purpura (ITP)

• Autoimmune thrombocytopenic purpura, or idiopathic

immune thrombocytopenic purpura, is an acute or chronic
bleeding disorder that results from immune destruction of
platelets by antiplatelet antibodies
 Etiology

• Autoantibodies of both immunoglobulin (Ig) G and IgM subclasses,

directed against a platelet-associated antigen, lead to destruction of
platelets in spleen, liver.
• Acute disorder more common in childhood, typically following viral
illness; has good prognosis with 80% to 90% recovering
uneventfully.
• Typically lasts 1 to 2 months.
• Chronic disorder (more than 6-month course) most common between
ages 20 and 40, three times more common in women, may last for
years or even indefinitely.
• May be associated with pregnancy or with development of systemic
lupus erythematosus or thyroid disease.
 Pathophysiology

• Precise cause is unknown, however, ITP is associated with viral

infections and occasionally medications such sulfur drugs.
• Conditions such as systemic lupus erythematosus (SLE) or
pregnancy can induce ITP thought to trigger the release of anti-
platelet autoantibodies that bind to the patient’s platelets.
• When the platelets are bound by the antibodies, the reticulo
endothelial system (RES) or tissue macrophage system ingests
the platelets, destroying them.
• The body attempts to compensate for this destruction by
increasing platelet production within the marrow.
 Clinical Manifestations

• The most common signs are bruising, petechiae, bleeding from nares and
gums, menorrhagia.
Diagnostic Evaluation
• Total blood cell count demonstrates platelet count less than 20,000/mm3
(acute ITP); 30,000 to 70,000/mm3 (chronic ITP); may also be
lymphocytosis and eosinophilia.
• Bone marrow aspirate shows increased numbers of young megakaryocytes,
• Assay for platelet autoantibodies
Medical Management
• Supportive care: use of platelet transfusions, control of bleeding.
• High-dose corticosteroids, I.V. immunoglobulins, parenteral anti-D (for
Rhesus positive patients with spleens), azathioprine (Imuran),
cyclophosphamide (Cytoxan), vincristine (Oncovin).
• Splenectomy removes potential site for sequestration and destruction of
platelets.
 Nursing Management

• Nursing Assessment
– Obtain focused history of bleeding episodes, including
bruising and petechiae, bleeding of gums, and heavy menses.
– Perform physical examination for signs of bleeding.
• Nursing Diagnosis
– Risk for Injury related to bleeding due to thrombocytopenia
• Nursing Interventions
– Minimizing bleeding by institute bleeding precautions.
– Patient education and health Maintenance by demonstrating
the use of direct, steady pressure at bleeding site if bleeding
does develop and Encourage routine follow-up for platelet
counts.
Disseminated intravascular coagulopathy (DIC)

• DIC is an acquired thrombotic and hemorrhagic syndrome

characterized by abnormal activation of the clotting cascade and
accelerated fibrinolysis.
• DIC, also called consumption coagulopathy and defibrination
syndrome, occurs as a complication of diseases and conditions that
accelerat clotting.
• This accelerated clotting process causes small blood vessel occlusion,
organ necrosis, depletion of circulating clotting factors and platelets,
and activation of the fibrinolytic system; which, in turn, can provoke
severe hemorrhage.
• Clotting in the microcirculation usually affects the kidneys and
extremities but may occur in the brain, lungs, pituitary and adrenal
glands, and GI mucosa.
• Other conditions, such as vitamin K deficiency and hepatic disease,
and anticoagulant therapy may cause a similar hemorrhage.
 Clinical Manifestations

• Abnormal bleeding (petechiae, hematomas, ecchymosis,

cutaneous oozing) without an accompanying history of a
serious hemorrhagic disorder
• Abdominal distention
• Coma, Dyspnea, Nausea Oliguria, Seizures, Severe muscle,
back, and abdominal pain, Shock, vomiting and Vertigo
 Diagnostic Evaluation

• Diminished Platelet count.

• Prolonged PT, PTT, and thrombin time.
• Decreased level of Fibrinogen.
• Increased level of Fibrin split (degradation) products.
• Increased level of D-dimer fibrin degradation product l.
• Decreased level of antithrombin III.
• Decreased level of Protein C.
 Medical Management

• Treat underlying disorder.

• Replacement therapy for serious hemorrhagic manifestations:
• Supportive measures including fluid replacement, oxygenation,
maintenance of BP and renal perfusion.
• Heparin therapy (controversial) inhibits clotting component of
DIC.
Nursing Management
• Nursing Assessment
– Monitor condition closely because these are seriously ill
patients
– Assess for signs of bleeding and thrombosis, including
 Nursing Diagnoses

• Risk for Injury related to bleeding due to thrombocytopenia

• Ineffective tissue perfusion related to ischemia due to
microthrombi formation
Nursing Intervention
• Minimizing Bleeding
• Promoting Tissue Perfusion
• Patient Education and Health Maintenance
• Explain the syndrome and its management to patient and
family members as part of reassurance and support during this
critical illness.
 Von willebrand's disease

• Von willebrand's disease is inherited autosomal dominant

bleeding disorder characterized by decreased level of von
Willebrand factor and prolonged bleeding time.
Pathophysiology
• Von Willebrand factor synthesized in vascular endothelium,
megakaryocytes and platelets; it enhances platelet adhesion as
first step in clot formation, also acts as carrier of factor VIII in
blood.
 Clinical Manifestations

• Mucosal and cutaneous bleeding

• Prolonged bleeding from cuts or after dental and surgical
procedures.
Diagnostic Evaluation
• Prolonged Bleeding time
• Decreased von Willebrand's factor
• Von Willebrand's factor multimers demonstrate defective von
Willebrand's factor in some types.
• Factor VIII is generally decreased.
 Medical Management

• Replacement of von Willebrand's factor and factor VIII using

clotting factor concentrates (Alphanate, Humate-P).
• Antifibrinolytic medications to stabilize clot formation
invasive surgeries.
• Estrogen and progesterone stimulate production of von
Willebrand's factor and Factor VIII.
 Nursing Management.

Nursing Assessment
• Obtain history of bleeding episodes such as menstrual flow.
Ask quantitative questions (such as, how many nosebleeds do
you have each year?) as patient may not realize his experience
is abnormal.
• Perform physical examination for signs of bleeding.
Nursing Diagnosis
• Risk for Injury related to bleeding due to decreased level of
von Willebrand's factor and factor VIII
 Nursing Interventions

• Institute bleeding precautions through:

– Avoid use of plain razor, hard toothbrush or floss.
– Avoid intramuscular injections, tourniquets, rectal
procedures or suppositories.
– Administer stool softeners as necessary to prevent
constipation.
– Restrict activity and exercise when platelet count less than
20,000/mm3 or when active bleeding occurs.
 CONT

• Monitor pad count and amount of saturation during menses;

administer or teach self-administration of hormones to suppress
menstruation as prescribed.
• Administer blood products as ordered. Monitor for signs and
symptoms of allergic reactions, anaphylaxis, and volume overload.
• Use topical hemostatic agents, such as absorbable gelatin (Gel
foam), oxidized cellulose (Surgicel), topical adrenaline, or
phenylephrine, if pressure and use of ice does not stop bleeding.
• Demonstrate the use of direct, steady pressure at bleeding site if
bleeding develops.
 Hemophilia

• A hereditary bleeding disorder that typically affects males,

hemophilia produces mild to severe abnormal bleeding.
• After a platelet plug develops at a bleeding site, the lack of
clotting factor prevents a stable fibrin clot from forming.
• Although hemorrhaging doesn’t usually happen immediately,
delayed bleeding is common.
• Two types of hemophilia exist:
 CONT

• i. Hemophilia A, or classic hemophilia

(deficiency or nonfunction of factor VIII)
• ii. HemophiliaB, or Christmas disease
(deficiency or nonfunction of factor IX). Severity
and prognosis of hemophilia vary with the
degree of deficiency and the site of bleeding.
• B. Causes
• Genetic inheritance: both types of hemophilia
inherited as X-linked recessive traits.
• Take Note 5. 4
• Hemophilia is inherited as an X-linked recessive trait. This
means that female carriers have a 50% chance of
transmitting the gene to a daughter, making
• her a carrier, and a 50% chance of transmitting the gene to a
son, who would be born with the disease

• C. Signs and symptoms

• i. Hematemesis (bloody vomitus)
• ii. Hematomas on the extremities, torso, or both
• iii. Hematuria (bloody urine)
• iv. History of prolonged bleeding after small cuts, surgery,
dental extractions, or trauma
• v. Joint tenderness
• vi. Limited ROM
• vii. Pain and swelling in a weight-bearing joint (such as the hip,
knee, or ankle)
• viii. Signs of decreased tissue perfusion: chest pain, confusion,
cool and clammy skin, decreased urine output, hypotension,
pallor, restlessness, anxiety, tachycardia
• ix. Signs of internal bleeding such as abdominal, chest, or flank
pain
• x. Tarry stools
• Severe hemophilia
• Excessive bleeding following circumcision (commonly the
first sign of the disease) Spontaneous or severe bleeding
after minor trauma
• Moderate hemophilia
• Occasional spontaneous bleeding, subcutaneous and I.M.
hematomas
• Mild hemophilia
• No spontaneous bleeding, Prolonged bleeding after major
trauma or surgery (blood may ooze slowly or intermittently
for up to 8 days following surgery)
• D. Diagnostic Findings
• Hemophilia A
• i. PTT is prolonged.
• ii. Factor VIII assay reveals 0% to 25% of normal factor VIII.
• iii. Platelet count and function, bleeding time, and PT are normal.
• Hemophilia B
• i. Baseline coagulation result is similar to that of hemophilia A, with
normal factor VIII.
• ii. Factor IX assay shows deficiency.
• E. Nursing Diagnoses
• i. Impaired gas exchange
• ii. Acute pain
• iii. Parental role conflict
• F. Treatment
• Hemophilia A
• 1. Administration of Factor VIII or
cryoprecipitate antihemophilic factor (AHF) and
lyophilized(dehydrated) AHF to encourage
normal hemostasis (arrest of bleeding)
• 2. Immediate notification of doctor
following injury, especially to the head, neck, or
abdomen
• Hemophilia B
• 1. Administration of purified factor IX to promote hemostasis
• 2. Immediate notification of doctor following injury,
especially to the head, neck, or abdomen
• Drug therapy (both types)
• Non-aspirin-containing analgesics to control joint pain
• G. Nursing Interventions and Rationales
• 1. Provide emotional support because hemophilia is a
chronic disorder.
• 2. Refer new patients to a hemophilia treatment center for
education, evaluation, and development of a treatment plan.
• 3. Refer patients and carriers for genetic counseling
to determine the risk of passing the disease to offspring.
• During bleeding episodes
• 1. Apply pressure to cuts and during epistaxis to stop
bleeding. In many cases, pressure is the only treatment
needed for surface cuts.
• 2. Apply cold compresses or ice bags and elevate the
injured part to control bleeding.
• 3. Give sufficient clotting factor or plasma, as
ordered, to promote hemostasis.
• 4. Administer analgesics to control pain. Avoid I.M.
injections because they may cause hematomas at the injection
site. Aspirin and aspirin containing medications are
contraindicated because they decrease platelet adherence and
may increase bleeding.
• If the patient has bled into a joint
• 1. Immediately elevate the joint to control bleeding.
• 2. Begin ROM exercises, if ordered, at least48 hours after
the bleeding has been controlled to restore joint mobility.
• 3. Don’t allow the patient to bear weight on the affected
joint until bleeding stops and swelling subsides to prevent
deformities due to hemarthrosis.
• After bleeding episodes and surgery
• 1. Watch for signs of further bleeding to detect and control
bleeding as soon as possible.
• 2. Closely monitor PTT. Prolonged times increase risk of bleeding.
• H. Teaching topics
• 1. Recognizing signs of severe internal bleeding
• 2. Notifying the primary care provider even after a minor injury
• 3. Wearing medical identification bracelet
• 4. Protecting a child from injury
• 5. Importance of medical follow-up
• 6. Risk of infection, such as hepatitis, from blood component
administration
• I. Diagnostic and therapeutic procedures of
the hematologic system
• These are the general procedures and
treatment modalities for blood disorders. They
include; Splenectomy, therapeutic apheresis,
therapeutic Phlebotomy, Blood and Blood
Component Therapy and transfusion.
• 5.5.6 Splenectomy
• The spleen is a fist-sized organ located in the upper left quadrant
of the abdomen. It includes a central white pulp where storage
and some proliferation of lymphocytes and other leukocytes
occurs, and a peripheral pulp involved in fetal erythropoiesis,
and later in erythrocyte destruction and the conversion of
hemoglobin to bilirubin. It may be surgically removed because of
trauma or to treat certain hemolytic or malignant disorders with
accompanying splenomegaly. A laparoscopic technique may be
used to remove a normal to slightly enlarged spleen in benign
conditions, such as idiopathic thrombocytopenic purpura,
hemolytic anemia, or sickle cell disease.
• Preoperative Management
• 1. Stabilization of preexisting condition:
• a. For trauma: volume replacement with I.V. fluids, evacuation of
stomach contents via nasogastric tube to prevent aspiration, urinary
catheterization to monitor urine output, assessment for
pneumothorax or hemothorax and possible chest tube placement.
• b. For hemolytic or malignant disorder with accompanying
thrombocytopenia: coagulation studies, administration of coagulation
factors (e.g., vitamin K, fresh-frozen plasma, and cryoprecipitate),
platelet and red cell transfusions.
• c. Preoperative pulmonary evaluation and teaching.
• d. For patient undergoing elective splenectomy, polyvalent
pneumococcal vaccine 10 to 14 days before procedure.
• Postoperative Management
• 1. Prevention of respiratory complications: hypoventilation and
limited diaphragmatic movement, atelectasis of left lower lobe,
pneumonia, left pleural effusion.
• 2. Monitoring for hemorrhage.
• 3. Administration of opioids for pain and observance for adverse
effects.
• 4. Monitoring for fever.
• a. Postsplenectomy fever mild, transient fever is expected.
• b. Persistent fever may indicate subphrenic abscess or hematoma
• 5. Monitoring daily platelet count: thrombocytosis (elevation of
platelet count) may appear a few days after splenectomy and may
persist during first 2 weeks.
• Potential Complications
• 1. Pancreatitis and fistula formation: tail of pancreas is anatomically close to
splenic hilum.
• 2. Hemorrhage.
• 3. Atelectasis and pneumonia.
• 4. Overwhelming postsplenectomy infection (OPSI) increased risk of developing a
life-threatening bacterial infection with encapsulated organisms, such as
Streptococcus pneumoniae, Neisseria meningitidis, or Haemophilus influenzae type B
• Nursing Diagnoses
• 1. Ineffective Breathing Pattern related to pain and guarding of surgical incision
• 2. Risk for Deficient Fluid Volume related to hemorrhage caused by surgery of
highly vascular organ
• 3. Risk for Injury (thromboembolism) related to thrombocytosis
• 4. Risk for Infection related to surgical incision and removal of the spleen
• 5. Acute Pain related to surgical incision
• Nursing Interventions
• Maintaining Effective Breathing
• 1. Assess breath sounds and report absent, diminished, or adventitious sounds.
• 2. Assist with aggressive chest physiotherapy and incentive spirometry.
• 3. Encourage early and progressive mobilization.
• Monitoring for Hemorrhage
• 1. Monitor vital signs frequently and as condition warrants.
• 2. Measure abdominal girth and report abdominal distention.
• 3. Assess for pain and report increasing pain.
• 4. Prepare patient for surgical reexploration if bleeding is suspected.
• Avoiding Thromboembolic Complications
• 1. Monitor platelet count daily, report abnormal result promptly.
• 2. If elevated, assess for possible thromboembolism.
• a. Assess skin color, temperature, and pulses.
• b. Advise patient to report chest pain, shortness of breath, pain, or weakness.
• 3. Report signs of thromboembolism immediately.
• Preventing Infection
• 1. Assess surgical incision daily or if increased pain, fever,
or foul smell.
• 2. Maintain meticulous hand washing and change
dressings using sterile technique.
• 3. Teach patient to report signs of infection (fever,
malaise) immediately.
• 4. Educate patient and family regarding OPSI, including
plan for postsplenectomy immunizations, recognition of
symptoms, use of prophylactic and standby antibiotics.
• Relieving Pain
• 1 Administer opioids or teach self-administration, as
prescribed and as necessary to maintain level of comfort.
• 2 Warn patient of adverse effects, such as nausea and
drowsiness; watch for hypotension and decreased respirations.
• 3 Teach the use of nonpharmacologic methods, such as the
use of music, relaxation breathing, progressive muscle
relaxation, distraction, and imagery to help to manage pain.
• 4 Document dosage of medications and response to
medication.
• 5 Make sure patient has analgesics for use post discharge.
• Patient Education and Health Maintenance
• 1. Teach care of incision.
• 2. Encourage to gradually increase activity according to
guidelines given by surgeon.
• 3. Advise proper rest, nutrition, and stress avoidance while
recovering from surgery.
• 4. Encourage follow-up as directed by surgeon and primary
care provider to maintain immunizations.
• 5. Encourage patient to seek prompt medical attention for
any infections and to contact health care provider immediately
for high fever.
• Activity
• Read about the following diagnostic and
therapeutic procedures of the hematologic
System and write down nursing cared before,
during and after the procedure.
• 1. Blood transfusion
• 2. Therapeutic apheresis
• 3. Therapeutic phlebotomy
• 4. Peripheral blood stem cell
transplantation