Catalog

Severe haemophilia A

No MMS code

Link to WHO Definition

Severe haemophilia A – http://id.who.int/icd/entity/96616786

Severe haemophilia A is a form of haemophilia A (see this term) characterised by a large deficiency of factor VIII leading to frequent spontaneous haemorrhage and abnormal bleeding as a result of minor injuries, or following surgery or tooth extraction. Severe haemophilia A accounts for around 40% of all cases of haemophilia A. The biological activity of factor VIII is below 1%. Transmission is X-linked recessive and the disorder is caused by mutations in the F8 gene (Xq28) encoding coagulation factor VIII.