Inhibitory hemophiliaD68.8

Author:Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 29.10.2020

Dieser Artikel auf Deutsch

Requires free registration (medical professionals only)

Please login to access all articles, images, and functions.

Our content is available exclusively to medical professionals. If you have already registered, please login. If you haven't, you can register for free (medical professionals only).


Requires free registration (medical professionals only)

Please complete your registration to access all articles and images.

To gain access, you must complete your registration. You either haven't confirmed your e-mail address or we still need proof that you are a member of the medical profession.

Finish your registration now

DefinitionThis section has been translated automatically.

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.

Occurrence/EpidemiologyThis section has been translated automatically.

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.

TherapyThis section has been translated automatically.

Treatment of the underlying disease.

Authors

Last updated on: 29.10.2020