Haemophilia Flashcards
haemophilia A is a factor ___ deficiency
VIII
presentation of haemophilia
haemarthrosis muscle atrophy muscle haematoma CNS bleed retroperitoneal bleed surgical bleeding bleeding after minor trauma
what type of joints fair worst in haemarthrosis
hinge joints
severe haemophilia is defined as coagulation factors activity at what %
<1%
true/false - haemophilia is x-linked
true - males affected, female carriers
at what age does haemophilia become apparent
6 months - 2 years
what blood test is of the intrinsic pathway and what change is observed in haemophilia
APTT - prolonged in haemophilia
what blood test is of the extrinsic pathway and hence unchanged in haemophilia
prothrombin time
true/false - foetal genetic testing can be done in utero to assess the presence of haemophilia
true
haemophilia is x-linked dominant/recessive
recessive
whats more common haemophilia a or b
A is 5 times more common
what is the platelet type of bleeding
muscosal pattern –> nose bleeds, brusing, purpura, menorrhagia and GI bleeds
what is the coagulation factor pattern of bleeding
articular, muscle haemotoma, CNS
prophylactic treatment for haem A
3 times weeky factor VIII infusions till maturity then stop
acute treatment of haem A
FFP or cryoprecipitate or factor VIII concentrate if available
desmopressin
tranexamic acid
prophylaxisin haem B
factor IX concentrate
complication more commonly seen in B than A
inhibitor development - body develops inhibitors to factor IX and it stops working
acute treatment of haem B
factor concetrate if available, cryoprecipitate or FFP if it’s not
tranexamic acid
management of both haem A and B (think orthopaedics)
DDVAP, splints, physio, analgesia, synovectomy, joint replacement
how does DDAVP work and what are its complications
releases factors from endothelial cells but can cause MI or hyponatraemia
what’s more common, von willebrand’s or haemophilia
von willebrand’s (1 in 200)
von willebrand’s shows platelet type or coagulation factor type bleeding
platelet type - mucosal
classificaition of von willebrand’s
type 1: quantative deficiency
type 2: qualitative
type 3: complete deficiency
treatment for von willebrand’s
vWF concentrate DDAVP tranexamic acid topic applications OCP
give 3 causes of thrombocytopenia
marrow failure, hypersplenism and marrow aplasia
treatment for thrombocytopenia
platelets
why does liver failure cause bleeding
cause it makes factors I, II, V, VII-XI. need vitamin K to do so
3 test results seen in liver failure thrombocytopenia
prolonged PT, APTT and reduced fibrinogen
true/false - vitamin K increased clotting factors within 30 mins
false - takes a few hours - so not good in acute
prophylaxis against haemorrhagic disease of the newborn
vitamin K injection in first few days
