FRCPath Haem Part 1 MCQs-Haemostasis 491

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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