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FRCPath Haem Part 1 MCQs-Haemostasis 491

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A 27-year-old woman with a history of menorrhagia and prolonged bleeding after dental extraction is referred to a haemophilia centre at 35 weeks of pregnancy. Her parents are first cousins. She has never had a thrombotic event and is not taking anticoagulants.

Investigation

Result

Hb

105 g/L

Platelets

248 × 10⁹/L

PT

18.2 s (prolonged)

APTT

68 s (prolonged)

Fibrinogen

4.1 g/L

Factor V activity

8 IU/dL

Factor VIII activity

34 IU/dL

VWF antigen

118 IU/dL

VWF activity

105 IU/dL

Mixing studies correct both the PT and APTT.


At 38 weeks, she presents in spontaneous labour. The obstetric team anticipates a possible emergency caesarean section. She has no active bleeding, and her factor VIII activity has risen to 62 IU/dL, while factor V remains at 8 IU/dL.


Which of the following is the single most appropriate management strategy?

Choose ONE best answer.


A. Administer desmopressin alone, as the pregnancy-associated rise in factor VIII should correct the combined deficiency.

B. Administer solvent/detergent-treated fresh frozen plasma (SD-FFP) at approximately 15–20 mL/kg

C. Administer recombinant factor VIII alone, targeting factor VIII above 100 IU/dL, because factor V deficiency does not independently increase surgical bleeding risk.

D. Administer recombinant activated factor VII (rFVIIa) alone as mandatory first-line treatment

E. Proceed without factor replacement because a factor V level above 5 IU/dL and a normal platelet count exclude clinically significant bleeding risk.


 
 
 

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