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Aktuelle Rheumatologie 2022; 47(06): 502-505
DOI: 10.1055/a-1876-2742
Übersichtsarbeit

Hemmkörperhämophilie: aktuelle Aspekte

Acquired Haemophilia: an Update
Marc Schmalzing
1   Rheumatology/Clinical Immunology, Department of Internal Medicine II, University of Würzburg, Würzburg, Germany
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Zusammenfassung

Die erworbene Hämophilie A stellt die häufigste Hemmkörperhämophilie dar. Sie kann idiopathisch, in Zusammenhang mit anderen Erkrankungen oder post partum auftreten. In einem Großteil der Fälle treten ausgeprägte Blutungszeichen auf, die lebensbedrohlich verlaufen können. Wichtige Differentialdiagnosen werden genannt. Die Diagnose wird ausgehend von der verlängerten aPTT durch Plasmamischversuche und den Bethesda-Test gestellt. Die Therapie erfolgt einerseits neben lokalen Maßnahmen zur Blutungskontrolle durch Gerinnungsfaktorpräparate sowie Tranexamsäure zur Blutstillung sowie andererseits durch Immunsuppression mit Glukokortikoiden, Rituximab und/oder Cyclophosphamid zur Elimination des inhibitorischen Autoantikörpers. Alternative Immunsuppressive Therapieoptionen für refraktäre Fälle werden aufgezeigt. Die Prognose scheint vor allem davon abhängig zu sein wie stark Faktor VIII initial reduziert ist.

Abstract

Acquired haemophilia A is the most common form of inhibitor-induced aquired haemophilia. It can occur idiopathically, in association with other diseases or post partum. In the majority of cases, there are pronounced bleeding signs that can be life-threatening. Important differential diagnoses are mentioned. The diagnosis is made on the basis of prolonged aPTT by plasma mixing tests and the Bethesda test. As treatment, local measures, coagulation factor preparations and tranexamic acid are used to stop bleeding, and immunosuppression with glucocorticoids, rituximab and cyclophosphamide to eliminate the inhibitory autoantibody. Further immunosuppressive treatment options to treat refractory cases are mentioned. Prognosis seems to depend mainly on the extent to which factor VIII is initially reduced.

Schlüsselwörter

Hemmkörperhämophilie - Diagnose - Therapie

Key words

aquired hemophilia - diagnosis - treatment


Publication History

Article published online:
18 August 2022

© 2022. Thieme. All rights reserved.

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