Disease basics

This section provides an overview of the clinical features associated with selected bleeding disorders.

Haemophilia A

Haemophilia A

Haemophilia A (HA) is an inherited bleeding disorder caused by the lack of functional coagulation factor (F) VIII, resulting in a defective clotting process and an increased tendency to bleed.

Haemophilia B

Haemophilia B

Haemophilia B (HB) is an inherited bleeding disorder caused by the lack of functional coagulation factor (F) IX, resulting in a defective clotting process and an increased tendency to bleed.

Haemophilia with inhibitors

Haemophilia with inhibitors

Alloantibody inhibitors to factor (F) VIII or FIX may develop in a subgroup of patients with haemophilia A (HA) or B (HB) and are a significant complication of haemophilia treatment. These inhibitors partially or completely neutralise the clotting activity of residual factor activity and render standard replacement therapy partially or completely ineffective.

Acquired Haemophilia A

Acquired
haemophilia A

Acquired haemophilia A (AHA) is a rare but potentially life-threatening autoimmune disorder attributable to autoantibodies toward an endogenous coagulation factor (F), most frequently FVIII.

Factor XIII congenital deficiency

Factor XIII congenital deficiency

Factor (F) XIII congenital deficiency (FXIII CD) is a rare autosomal recessive bleeding disorder that is the result of an impaired production of FXIII, the terminal enzyme in the blood coagulation cascade that stabilises the already formed blood clot during the haemostatic process.

Factor VII congenital deficiency

Factor VII congenital deficiency

Factor VII congenital deficiency (FVII CD) is an inherited bleeding disorder caused by the lack of coagulation factor (F) VII, resulting in a defective clotting process and an increased tendency to bleed.

Glanzmanns Thrombosthenia

Glanzmann’s thrombasthenia

Glanzmann’s thrombasthenia (GT) is a rare autosomal recessive platelet function disorder caused by a quantitative or qualitative defect in platelet membrane glycoprotein IIb/IIIa, which functions as a receptor for fibrinogen and is involved in platelet aggregation and fibrin clot retraction.

HQMMA/CH/0217/0024; HQMMA/CH/0916/0170; HQMMA/NNG/0517/0163; HQMMA/NNG/0517/0164; HQMMA/NNG/0517/0165; HQMMA/NNG/0517/0166; HQMMA/NNG/0517/0167