bleeding disorders Flashcards
what is affected in platelet type bleeding ?
mucosal epistaxis purpura menorrhagia GI
what is affected by coagulation bleeding ?
articular
muscle heamatoma
CNS
what is the epidemiology of haemophilia A and B ?
X-linked A - 1 in 10,000, B - 1 in 60,000 <1% severe 1-5% moderate 5-30% mild
what are clinical features of haemophilia ?
haemarthrosis muscle haematoma CNS bleeding retroperitoneal bleeding post-surgical bleeding
what is haemophilia A ?
deficiency of factor 8
what is haemophilia B ?
deficiency of factor 9
what are clinical complications of haemophilia ?
synovitis
chronic haemophilic arthropathy
neurovascular compression
stroke
how is haemophilia diagnosed ?
clinical
prolonged APTT, normal PT
reduced factor 8/9
genetic analysis
how do you treat haemophilia ?
coagulation factor replacement
DDAVP - Desmopressin
tranexamic acid
prophylaxis
splints, physio, analgesi, synovectomy, joint replacement
what are treatment complications of haemophilia ?
viral infection - HIV, HBV
inhibitors - anti F8 Ab
DDAVP - MI, hyponatraemia in babies
what is Von Willebrand disease ?
autosomal dominant
quantitative and qualitative abnormality of vWF
common 1/200
produces platelet type bleeding
what are the different types of Von Willebrand disease ?
type 1 - quantitative deficiency
type 2 - qualitative deficiency determined by site of infection
type 3 - severe deficiency
how do you treat Von Willebrand disease ?
vWF concentrate DDAVP tranexamic acid topical applications OCP
what are examples of acquired bleeding disorders ?
thrombocytopenia liver failure renal failure DIC drugs
what causes thrombocytopenia ?
decreased production - marrow failure, aplasia, infiltration
increased consumption - immune ITP, non-immune DIC, hypersplenism
