Haematology + Oncology

Question 1

Features of haemophilia C

Answer 1

• Factor XI deficiency
• Equal incidence in males and females (autosomal recessive, though carriers may exhibit some symptoms)
• Less common than both haemophilia A (most common) and haemophilia B
• Bleeding risk in hemophilia C is not always influenced by the severity of the deficiency (contrary to haemophilia A & B)
• Unpredictable bleeding tendency
• Usually presents with bleeding after signficant trauma or surgery, increased bruising, epistaxis, menorrhagia, or postpartum haemorrhage
• Spontaneous bleeding rarely occurs
• Haemarthroses are not seen
• Can present in childhood or as an adult
• Treatment is with supportive care, hepatitis vaccination, pre-operative fresh frozen plasma or recombinant factor XI

Question 2

Post splenectomy blood film features

Answer 2

Howell- Jolly bodies
Pappenheimer bodies
Target cells
Irregular contracted erythrocytes

Question 3

Heritable causes of DVT from most to least common

Answer 3

• Factor V Leiden
• Prothrombin G20210A variant
• Protein C deficiency
• Protein S deficiency
• Antithrombin deficiency

Question 4

Flow cytometry results in PNH

Answer 4

CD55 and CD59 negative cells