Haemostasis tutorial

1. A 30 year old woman presents with pallor, bruising petechiae. She is afebrile
but is noted to have mild splenomegaly. The following results are obtained:

Hb 8g/dL (11.5 – 16.5)

WCC 27 x 10x9/l (4 – 10)
Plt 22 x 109/l (150 – 400)
APTT 45 sec (26 – 35)
PT 19 sec(12 – 19)
D-dimer 2.00 mg/L (<0.25)
Fibrinogen 1.2 g/L (1.8 – 4.0)

The bone marrow appearance shows large blasts with numerous Auer
rods.
What is the most likely current diagnosis?
What is the treatment plan?

2. A 30 year old woman presents with reduced urine output and lethargy
several days after an episode of bloody diarrhoea. Investigations reveal:

Creat 253mmol/L (65-135)

Hb 10.2 g/dL
MCV 83 fL
WCC 7.2 x 109/l
Plts 76 x 109/l

The blood film shows fragmented fragmented RBCs

a. Which of the following is the most likely diagnosis?

b. What is the treatment plan?

3. A 23 year old female presents with complaints of “spots on her legs”. She
has been well. FBC shows Hb 14.5g/dl, WBC 8.9 x109 /l, normal
differential, platelets 13x109/l. The peripheral blood film is significant
only for thrombocytopenia.

a. What is the probable diagnosis?

b. What secondary conditions do you need to exclude?
c. What is the most appropriate treatment?

The patient is treated and responses initially for about 3 months but she keeps
relapsing with thrombocytopenia.
d. What treatment options are available for this patient?

4. A 42year old woman is brought to the emergency room with sudden onset
aphasia and right sided hemiparesis. History from the husband says she
 has been feeling unwell these past 2 days but no fever, cough or diarrhea.
She is hypertensive on hydrochlorothiazide and her blood pressure is
normal. FBC done on routine examination was normal.

On examination she has complete motor aphasia and decreased power 2/5
on right upper and lower limbs. There are no petechiae or ecchymoses on
her skin. FBC: Hb 9g/dl, WBC 7.3x109/l, platelets 15x109/l; peripheral film
shows schistocytes and confirms thrombocytopenia.
a. What is the most probable diagnosis?
b. What further investigations would you do?
c. What would be your management of this patient?

5. A 23 year old man has been sent by the dentist because he had a wisdom
tooth extraction done about a month ago. He had excessive bleeding with
the procedure but developed a large clot which continued to ooze for 2
days. He was reviewed by the dentist and the wound was cleaned and
packed but he had bleeding again and vomited blood. The wound was
stitched and he was sent for further evaluation.
a. What pertinent history questions would you need to illicit?
b. What investigations would you perform?
c. What is the most probable diagnosis?
d. What precautions would you advice for future dental surgery?

6. The following results are obtained from a 40 year old man prior to elective
hernia surgery who gives a history of excessive bleeding after dental
extraction.

Bleeding time 16 mins (2 – 9mins)

PT 12 secs (12 – 14secs)
INR 1.0
APTT 48 secs (26 – 35secs)
Fibrinogen 2.4 g/L (1.8 – 4.0)

Mixing the patient’s plasma with an equal volume of normal plasma the
APTT is 27s (26-35s)

Which one of the following is the most likely diagnosis?

a. Factor VII deficiency

b. Recent aspirin ingestion
c. Anti phospholipid syndrome
d. Von Willebrand’s disease
e. Haemophilia B

7. A 46-year-old woman was admitted with a 7-day history of malaise, lethargy,

bloody diarrhoea, haematuria and acute dyspnoea. She is a cattle worker on a
farm. She is pyrexial and appears jaundice. There is evidence of pulmonary
oedema with a fine petechial rash over her face. Her blood pressure is elevated
at 190/90. There is no neurological abnormality identified. Urinalysis shows
 red cell casts with blood and protein. The following investigations are listed
below:

haemoglobin 6.2 g/L (11.5–16.5)

platelet count 43 × 109/L (150–400)
prothrombin time18.0 s (11.5–15.5)
activated partial thromboplastin time 56 s (30–40)

serum sodium 140 mmol/L (137–144)

serum urea 37.0 mmol/L (2.5–7.0)
serum creatinine 480 µmol/L (60–110)
serum total bilirubin 93 µmol/L (1–22)
serum lactate dehydrogenase 2630 U/L (10–250)

Blood film: shistocytes, helmet cells and thrombocytopenia

What is the most likely underlying diagnosis?
a. DIC
b. TTP
c. atypical HUS
d. HUS
e. PNH

8. Which of the following statements about the clinical manifestation of TTP is

FALSE?

a. Neurological abnormalities occur commonly

b. A microangiopathic haemolytic anaemia is found
c. Thrombocytopenia may occur
d. Acute kidney injury occurs commonly
e. ADAMTS13 factor levels are low

9. A 36 year old black female presents with sudden onset left hemiparesis, a
purpuric rash and confusion. Investigations reveal a microangiopathic
haemolytic anaemia, thrombocytopenia without evidence of coagulopathy or
renal failure. ADAMTS13 activity and levels are decreased. There is an
absence of ADAMTS13 autoantibody levels.
What is TRUE regarding the correct interpretation of the diagnostic tests?

a. TTP likely, hereditary, ADAMTS13 genetic mutations are required

b. atypical HUS likely
c. HUS likely
d. TTP confirmed, acquired, ADAMTS13 genetic mutations are not
required
e. drug induced TTP likely, ADAMTS13 genetic mutations are not
required
10. What is the function of ADAMTS13?

a. Prevents di-sulfide bridging of vWF multimers

b. Cleaves A2 domain of large vWF multimers
c. Cleaves B6 domain of large vWF multimers
d. Decreases synthesis of vWF multimers by transcriptional regulation
e. allosterically inhibits the function of vWF

11. A 7 year old girl requiring a tonsillectomy and adenoidectomy undergoes

pre-operative laboratory testing by her surgeon revealing a prolonged
activated partial thromboplastin time (PTT) of 150 seconds (normal
range 22-36 seconds). Her prothrombin time (PT) is normal. She had
previously had 2 dental extractions that were uneventful. She otherwise
has no personal or family history of bleeding. Which of the following is
most likely:
a. Factor VIII deficiency
b. Factor IX deficiency
c. Factor VII deficiency
d. Factor XI deficiency
e. Factor XII deficiency

12. You are evaluating a 12 year girl who was admitted to the hospital for
anemia (hemoglobin concentration of 85 gm/L) who has had significant
vaginal bleeding with the onset of menarche 3 weeks ago. Her family
history includes several females who were diagnosed with von
Willebrand disease (VWD). Which of the following tests are necessary to
detect the presence of type 2A VWD?

a. Von Willebrand factor (VWF) antigen concentration

b. Ristocetin cofactor activity
c. Factor VIII activity
d. VWF multimer analysis
e. Ristocetin-induced platelet aggregation with low dose ristocetin

13. You are seeing a 12 year boy old with easy bruising and recurrent
epistaxis as a second opinion. He is active in a variety of sports, however
his mother feels that his bruising is excessive. His pediatrician sent the
following laboratory tests all of which are normal: WBC, Hemoglobin,
platelet count, PT, PTT. Another hematologist ordered the following all of
which were normal: VWF Ag, ristocetin cofactor activity, factor VIII
activity, factor XIII activity, and platelet aggregation studies. Which of the
following physical exam findings would be most informative:

a. Petechiae where the blood pressure cuff was placed.

b. Hypermobility of the finger joints.
c. Palpable bruises over the tibial surface.
d. A conjunctival hemorrhage
e. Albinism.

14. A 10 day old male is being seen in the emergency room due lethargy and
poor feeding. Her anterior fontanel is full. A CT scan demonstrates an
intraparenchymal hemorrhage. Coagulation tests are ordered with the
following results: PT—37 seconds (normal 9.7-11.2 seconds) and a PTT of
66 seconds (normal 22-36 seconds). This child may have which of the
following factor deficiencies:
a. Factor VII.
b. Factor VIII.
c. Factor IX.
d. Factor X.
e. Factor XI.

15. A 5 year old male presents with fulminant acute hepatic failure. He is
noted to be bleeding from his gums, nose and has hematochezia. This
patient’s bleeding is most likely due to which of the following
combinations:

a. Deficiency of fibrinogen, factor VII and factor II.

b. Thrombocytopenia and factor XI deficiency.
c. Factor VIII, IX and XI deficiency.
d. Factor V and VIII deficiency.
e. Low levels of von Willebrand factor and factor VIII.

16. A 10 day old male is being seen in the emergency room due lethargy and
poor feeding. Her anterior fontanel is full. A CT scan demonstrates an
intraparenchymal hemorrhage. Coagulation tests are ordered with the
following results: PT—37 seconds (normal 9.7-11.2 seconds) and a PTT of
66 seconds (normal 22-36 seconds). This child may have which of the
following factor deficiencies:

a. Factor VII.
b. Factor VIII.
c. Factor IX.
d. Factor X.
e. Factor XI.