Der Gerinnungsfaktor XIII – Pathophysiologie, Klinik und Therapie von Mangelzuständen

Zusammenfassung

Die Transglutaminase Faktor XIII (FXIII) besitzt ein vielfältiges Wirkspektrum, das zentrale Funktionen in der sekundären Hämostase einschließt. Multifaktoriell bedingte FXIII-Mangelzustände können u. a.mit Aborten, Wundheilungsstörungen und lebensbedrohlichen Koagulopathien assoziiert sein. Die vorliegende Übersichtsarbeit beschreibt die physiologischen Funktionen von FXIII sowie Pathophysiologie, Diagnostik und Therapieoptionen von FXIII-Mangelzuständen.

Abstract

The complex activity of the transglutaminase factor XIII (FXIII) comprises central functions in secondary hemostasis. Congenital or acquired FXIII deficiencies may be associated with habitual abortions, impaired wound healing, coagulopathy and fatal hemorrhage. The present review describes physiological functions of FXIII, as well as pathophysiology, diagnostic and therapeutic options of FXIII deficiencies.

Schlüsselwörter:

Faktor XIII - Gerinnung - Hämostase - Faktor-XIII-Defizit - Koagulopathie

Keywords:

factor XIII - coagulation - hemostasis - factor XIII deficiency - coagulopathy

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