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Pathology

Haemophilia A: low levels of factor VIII 
Haemophilia B: low levels of factor IX

Aetiology

X linked genetic defect in the responsible genes (male only disease, females are carriers)

Signs

Haemarthrosis, spontaneous bleeds into muscles and soft tissue, haematuria, GI bleed.

Symptoms

Bleeding and bruising

Investigations

Clotting screen: Prolonged APTT, normal PT, normal vWF,
Decreased Factor VIII (Haemophilia A)
Decreased Factor IX  (Haemophilia B)

Treatment

Medical: Deficient Factor replacement and Desmopressin increases factor VIII

Prognosis

Good with prophylactic and replacement recombinant  factor therapy. 
Fatal hemorrhage in the absence of factor replacement .
Chronic arthropathies, contraction of Hepatitis A, B, C, HIV, Risk of variant CJD 


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