MEQ

Case 1
Jake Harris is a 70 kg, 22 year old law student working part-time as a
labourer on a building site, who presents with a tender, swollen and painful
right knee joint, following a heavy fall at work. Physical examination is
otherwise unremarkable. Jake has no previous history of bleeding
problems, nor has he had any surgical or dental procedures.
Question 1.1
Give at least three plausible causes for his tender, swollen knee.

Question 1.2
Which two sets of ligaments are the major stabilisers of the knee joint and where are
they located with respect to the knee joint (specific attachments not required)?

You order coagulation studies that show:

Prothrombin time: 13 seconds (NR 11-16 sec)
Activated partial thromboplastin time (APTT): 60 seconds (NR 30-40 sec)
Platelet count: 200 x 109/L (NR 150-400 x 109/L)
Question 1.3
Name at least two plausible causes of prolonged APTT and give a brief explanation
for each.

Factor assays reveal Factor IX 95%, Factor V 75%, Factor VIII 15%, Factor
XII 78%. This is mild haemophilia presenting with a haemarthrosis
secondary to trauma.
Question 1.4
List at least two investigations and two management issues which should be
explored.

Question 1.5
What treatment would you initiate to prevent further bleeding into the joint?

Question 1.6

a) Give two mechanisms by which von Willebrand factor contributes to normal

haemostasis.
b) Explain the consequences of the failure of at least one of these mechanisms for
normal haemostasis.

Question 1.7
Describe three strategies that might have been adopted to prevent (or at least
minimise the risk) of injury. For each one, you must state the level of prevention.
(Hint: Consider host, environment, and vector/vehicle)

Mr Burke asks you how common is haematological disease in the Australian

community.
Question 1.8
a) Name ONE source (other than Medline) that you believe is most likely to help you
find out about the frequency of haematological disease in the Australian community.
Only your first response will be marked.
b) What are the criteria by which you will judge the credibility and legitimacy of this
source. Give two.

SBA
Case 1
Samuel Irvine, an 18 month old haemophiliac, presents with a right knee
haemarthrosis to the Emergency Department of a district hospital. He has
had a previous admission to the same hospital for the same problem.
Question 1.1
In children, the clinical finding most suggestive of an inherited coagulation factor
defect would be:
A) Bruises on both shins from an unknown cause
B) Bruises on his chest wall from lying on a toy
C) Sudden onset of petechia
D) Recurrent nose bleeds lasting for 1 minute

Question 1.2
Which of the following screening tests is abnormal in patients with haemophilia A?
A) Platelet count
B) Activated Partial Thromboplastin Time (APTT)
C) Prothrombin Time (PT)
D) Thrombin Time (TT)

Question 1.3
The factor VIII gene encodes a protein of approximately 2,300 amino acids. Given
that the gene spans 186 kilobases of the X chromosome, it is accurate to say that
the majority of the DNA in the factor VIII gene is organized into:
A) Exons
B) Introns
C) Protein encoding regions
D) RNA encoding regions

Question 1.4
Circulating Von Willebrands factor is a protein that influences coagulation by acting
to:
A) Degrade factor VIII by proteolysis
B) Convert factor VIII to its active form
C) Stabilize factor VIII in the circulation
D) Cross-link factor VIII to its receptors

Question 1.5
In haemophilia:
A) The molecular mutation is the same in all patients
B) The family history is negative in over 50% of patients
C) In families with informative RFLP analysis, intragenic markers are more accurate
than extragenic markers
D) The mother of a severe haemophiliac is unlikely to be a carrier if the family
history is negative

Case 2
Max Friedrichs is an alert three year old boy who has been recently
diagnosed with haemophilia A.
Question 2.1
A diagnosis of haemophilia A is suggested by:
A) A reduced factor IX level
B) A factor XIII level of <10%
C) Reduced levels of von Willebrand factor
D) A long activated partial thromboplastin time (APTT)

Question 2.2
The replacement product of choice for a newly-diagnosed boy with Haemophilia A is:
A) Recombinant factor VIII
B) Cryoprecipitate
C) AHF (anti-haemophilia factor)
D) Fresh frozen plasma

Question 2.3
The prothrombin time (PT) is prolonged in association with:
A) Blood transfusion
B) Heparin therapy
C) Severe thrombocytopenia
D) Warfarin therapy

Question 2.4
In the coagulation cascade, which of the following factors, when activated, cleaves
prothrombin to thrombin?
A) Factor XI
B) Factor X
C) Factor IX
D) Factor VIII

Question 2.5
A female carrier for classical haemophilia marries a close relative with classical
haemophilia. The risk of a female infant being affected with haemophilia is:
A) 1.0
B) 0.5

C) 0.25
D) zero

Question 2.6
Non-steroidal drugs, such as aspirin, reduce platelet aggregation because they:
A) Inhibit megakaryocyte formation in bone marrow
B) Cause immune thrombocytopenic purpura
C) Block GPIIIa receptors on platelets
D) Inhibit the formation of thromboxane A2 in the platelets

Case 3
Joan Thomas is an 82 year old woman with myelodysplasia who receives
regular blood transfusion.
Question 3.1
For the correction of anaemia the blood product of choice is:
A) Whole blood
B) Intragam
C) Packed red blood cells
D) Fresh frozen plasma

Question 3.2
Which one of the following is the most common complication of blood transfusion?
A) Acute haemolysis from ABO incompatible blood transfusion
B) Human immunodeficiency virus infection
C) Hepatitis C infection
D) Human T-cell lymphotrophic virus infection

Question 3.3
Which one of the following individuals would be excluded from being a blood donor?
A) A donor who lived in the UK in the 1980s
B) A donor who travelled to New Guinea this year
C) A New Zealand Maori living in Australia
D) A donor with medicated chronic hypertension

Question 3.4
With respect to red blood cells prepared for transfusion:
A) The shelf life is 35 days
B) Storage is at room temperature
C) A viral inactivation step is part of the preparation
D) Each pack is tested for drug metabolites

Case 4
John Berkley is a strong, tall and slim University Professor. He enjoys
walking in the park (though he has a limp). He has a history of bleeding into
joints with minimal trauma.
Question 4.1
A diagnosis of haemophilia A is suggested by:
A) A reduced factor IX level
B) A long activated partial thromboplastin time (APTT)
C) Reduced levels of von Willebrand factor
D) A factor XIII level of <10%

Question 4.2
The prothrombin time (PT) is prolonged in association with:
A) Severe thrombocytopenia
B) Warfarin therapy
C) Blood transfusion
D) Heparin therapy

Question 4.3
The replacement product of choice for a newly-diagnosed boy with Haemophilia A is:
A) Cryoprecipitate
B) Fresh frozen plasma
C) Recombinant factor VIII
D) AHF (anti-haemophilia factor)

Question 4.4
Non-steroidal drugs, such as aspirin, reduce platelet aggregation because:
A) They inhibit megakaryocyte formation in bone marrow
B) They inhibit the formation of thromboxane A2 in the platelets
C) They cause immune thrombocytopenic purpura
D) They block GPIIIa receptors on platelets

Question 4.5
The risk of viral transmission is lowest for which of the following blood products:
A) Platelet concentrates
B) AHF (anti-haemophilia factor)
C) Leucocyte depleted red blood cells

D) Cryoprecipitate

Question 4.6
A female carrier for classical haemophilia marries a close relative with classical
haemophilia. The risk of a female infant also being a carrier is:
A) zero
B) 0.25
C) 0.5
D) 1.0

Question 4.7
A female carrier for classical haemophilia marries a close relative with classical
haemophilia. The risk of a female infant also being a carrier is:
A) zero
B) 0.25
C) 0.5
D) 1.0

Case 5

Question 5.1

Question 5.2

Question 5.3

Question 5.4

10

Question 5.5

11

Case 6

Question 6.1

Question 6.2

Question 6.3

12

Question 6.4

13

Question 6.5

14