Spontane Faktor-VIII-Inhibitoren

Zusammenfassung

Als spontane (nichthämophile) Faktor-Vlll-lnhibitoren werden Antikörper gegen Faktor VIII bei zuvor gerinnungsnormalen Patienten bezeichnet. Meist ist der F-Vlll-Mangel schwer und dementsprechend die Blutungsneigung ausgeprägt. Die Pathogenese ist heterogen. Laborchemisch findet sich typischerweise eine verlängerte aPTT und ein isolierter F-Vlll-Mangel. Die Antikörper sind polyklonal und zumeist Typ-Il-Inhibitoren. Die Therapie gliedert sich in die Beherrschung der akuten Blutung (hochkonzentrierte humane F-Vlll-Konzentrate, porciner F VIII, aktivierte Prothrombinkomplexpräparate, rekombinanter F Vlla, evtl. zusätzlich Plasmapherese und Immunadsorption - zur Reduktion des Inhibitors) und in die Langzeitreduktion des Titers mit Immunsuppressiva.

Schlüsselwörter

Spontane Faktor-VlIl-lnhibitoren - Hemmkörperhämophilie A - Diagnose - Therapie

• LITERATUR

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