INHERITED BLEEDING DISORDERS Flashcards
inherited bleeding disorders can affect
- coagulation cascade
- platelets
- both
haemophilia
too little clot formed
thrombophilia
too much clot formed
factor VII def
haemophilia/ A
Factor IX def
christmas disease
Haemophilia B
reduced factor VII and reduced platelet aggregation
von willebrands disease
DDAVP
desmopressin
releases factor VIII to boost factor VII levels and clotting ability
tranexamic acid
inhibitor of fibrinolysis - keeps any clot that is formed
Haemophilia A and B affects who
sex linked recessive - males affected, females carriers
defect gene on X chromosome
management of haemophilia A
severe and mod - recomb factor VIII
mild and carriers - DDAVP/ tranexamic acid
management of haemophilia B
severe and mod - recomb factor IX
mild and carriers - recomb factor IX
Von wilebrand disease affects who
autosomal dominant
not transmitted on X chromosome
both sexes equally affected
mild types of VW
1 and 2 (dominant)
severe types of VW
type 3 (recessive)
management of VW
severe and mod - DDVAP
mild and carriers - oral tranexamic acid