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Hamostaseologie 1996; 16(03): 171-173
DOI: 10.1055/s-0038-1656652
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Schattauer GmbH

Erworbene Inhibitoren gegen Faktor IX

Inge Scharrer
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Publikationsdatum:
23. Juli 2018 (online)

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Zusammenfassung

Spontan erworbene Faktor-IX-Hemmkörper treten sehr selten auf, wesentlich seltener als spontan erworbene Faktor-Vlll-Hemmkörper. Die durch sie verursachte Blutungsneigung ist ähnlich derjenigen, die durch Faktor-Vlll-Hemmkörper induziert werden kann. Auch die Grundkrankheiten sind ähnlich denen, die bei den spontanen Faktor-Vlll-Hemmkörpem vorliegen.

Die Diagnose erfolgt mit Hilfe der Bethesda-Methode. Lupus-Antikoagulanzien sollten vorher ausgeschlossen werden. Faktor-IX-Hemmkörper sind nicht so ausgeprägt temperatur- und zeitabhängig wie Faktor-Vlll-Inhibitoren. Sie weisen keine komplexe Kinetik auf. Meist sind es Immunglobuline der IgG-Klasse 1-4. Die Blutstillungstherapie kann mit Feiba® oder rekombinantem Faktor Vila erfolgen. Durch Plasmapheresen kann die Titerhöhe reduziert werden. Zur Elimination empfiehlt sich eine immunsuppressive Therapie, zum Beispiel eine Kombination von Cyclophosphamid und Kortikosteroiden.

Schlüsselwörter

Faktor-IX-Hemmkörper - postpartale Hemmkörper - Bethesda-Methode - Blutstillungstherapie - Eliminationstherapie
 

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