abnormal coagulation

Question 1

what is haemophilia

Answer 1

X-linked recessive disorder of coagulatio

Question 2

what factors are affected in haemophilia A and B

Answer 2

A: factor VIII
B: factor IX

Question 3

features of haemophilia

Answer 3

haemoarthroses: big joints
haematomas
prolonged bleeding after surgery or trauma

Question 4

blood tests in haemophilia

Answer 4

prolonged APTT
bleeding time, thrombin time and prothrombin time normal

Question 5

management of haemophilia

Answer 5

A: recombinant FVIII
B: recombinant FIX

Question 6

most common inherited bleeding disorder

Answer 6

von willebrand’s disease

Question 7

presentation of von willebrand’s disease

Answer 7

epistaxis and menorrhagia common
less commonly haemoarthroses and haematomas

Question 8

investigations for von willebrand’s disease

Answer 8

prolonged bleeding time
APTT may be prolonged
factor VIII levels moderately reduced
defective platelet aggregation with ristocetin

Question 9

management of von willebrand disease

Answer 9

tranexamic acid for mild bleeding
desmopressin
factor VIII concentrate