Inherited bleeding disorders

Question 1

what factor is lacking in haemiphilia A

Answer 1

factor 8

Question 2

what factor is lacking haemophilia B

Answer 2

factor 9

Question 3

what mode of inheritance is seen in haemophilia

Answer 3

X-linked recessive

Question 4

how do you measure the intrinsic pathway

Answer 4

APTT

Question 5

what blood result would you see in haemophilia A and B

Answer 5

isolated prolonged APTT
normal prothrombin time
normal fibrinogen

Question 6

what pathway are factors 8 and 9 involved in the coagulation cascade

Answer 6

intrinsic pathway

Question 7

what are the clinical features of haemophilia

Answer 7

prolonged bleeding/rebleeding/excess bleeding
unusual surgical bleeding
bleeding with IM injections/vaccinations

Question 8

when could you use recombinant synthetic factor in haemophilia

Answer 8

• on demand for bleeding episodes
• targeted prophylaxis for surgery/dental work
• primary prophylaxis for those with severe/moderate disease

Question 9

what is the commonest inherited bleeding disorder

Answer 9

von willebrand disease

Question 10

what is the rough pathology associated with von willebrand disease

Answer 10

defective platelet adhesion

Question 11

what factor does von willebrand factor bind

Answer 11

factor 8

Question 12

what are the clinical features of von willebrand disease

Answer 12

mucosal bleeding
easy bruising
dental bleeding
epistaxis
menorrhagia
surgical and post0traumatic bleeding