Inhibitory hemophilia D68.8

Inhibitory haemophilia is a rare immunological disease in which antibodies against individual coagulation factors inhibit the activity of these factors and can lead to extreme bleeding.

These diseases can occur in haemophiliacs who, as part of a substitution therapy, form antibodies against the coagulation factors and inactivate them. It is observed in about 6-13% of patients with haemophilia A and in 2-4% of patients with haemophilia B.
This disease is often detected in hemophiliacs as early as childhood. It is characterized by a tendency to bleed despite substitution therapy. The basis is the extent of the factor VIII or factor IX mutation.
The more the mutations differ from the normal factors, the greater the probability that the normal factors will be identified as foreign.

The constellation with dermatitis herpetiformis, bullous pemphigoid and pregnancy is rarer.