Erworbene Inhibitoren gegen Faktor XIII

Zusammenfassung

Antikörper gegen FXIII sind extrem selten: Bisher wurden zwei Patienten mit angeborenem FXIII-Mangel und 21 vorher gerinnungsnormale Patienten beschrieben, die Hemmkörper gegen FXIII entwickelten. Häufigster Auslöser für erworbene FXIII-Hemmkörper, die eine defekte Fibrinvernetzung verursachen, ist die Langzeitanwendung bestimmter Medikamente. Die Blutungssymptomatik zeigt das gesamte Spektrum des schweren FXIII-Mangels. Sofern ein Medikament als Ursache des Hemmkörpers in Frage kommt, ist dieses sofort abzusetzen. Weiterhin sollte versucht werden, durch hochdosierte Gabe eines FXIII-Konzentrates den Inhibitor zu überspielen und damit eine Blutstillung zu erzielen

Schlüsselwörter

Faktor-Xlll-Inhibitor - Pathogenese - Blutungssymptomatik - Therapie

• LITERATUR

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