[Congenital haemorrhagic diseases].

Congenital haemorrhagic diseases are the consequences of haemostasis dysfunction or defects. Among primary haemostasis disorders, von Willebrand disease is the most frequent. Hopefully the type 1 von Willebrand disease, which affects 85% of patients, is generally a mild haemorrhagic disorder. However it may induce some troubles during the everyday life. On the other side, haemophilia is the most frequent congenital coagulation disorders. Haemophilia A is due a factor VIII deficiency while haemophilia B is due to a factor IX deficiency. Haemophilia is mainly a musculoskeletal disease. Recurrent joint bleedings may lead to a severe disabling arthropathy. The organization of care for hemophilia is managed by haemophilia treatment centers. Other rare bleeding disorders may lead to the same complications but requires specific treatments.