Hemophilia its causes,classification,diagnosis,complication and newest treatment

•          Caused by an absence or decreased amount of a procoagulant –

•          VIII -Hemophilia A affects ~ 1:5000 males

•          XI -Hemophilia B affects ~ 1:30000 males

•          XI –Hemophilia C – Rare /Ethnicity

Woman can have hemophilia

•          Lyonization of the normal X chromosome

•          Turner syndrome ( XO)

•          Father with hemophilia/ mom as a carrier

•          vW type 2 N ( Normandy)

•          Laboratory findings: Rolonged PTT

•          Normal PT and thrombin time

•          Decreased level of factor VIII

Complications 
•          Joint derormities.

•          Inhibitors to factor VIII

•          Hepatitis

•          AIDS

•          Management

General: dental hygiene, avoid injury,avoid aspirin,replacement therapy with surgery

•          Replacement therapy:

•          Fresh frozen plasma

•          Factor VIII concentrates

•          Intranasal form of desmopressin acetate

•            

Prophylaxis

•          Factor VIII concentrates (20-40IU/Kg) every 3 days with severe form