Semin Thromb Hemost 1996; 22(5): 419-425
DOI: 10.1055/s-2007-999041
Copyright © 1996 by Thieme Medical Publishers, Inc.

Factor XIII Deficiency: Pathogenic Mechanisms and Clinical Significance

Rudolf Egbring* , Arno Kröniger* , Rainer Seitz
  • From the *Department of Hematology/Oncology, Philipps-University Hospitals, Marburg, Germany and the
  • †Department of Hematology and Transfusion Medicine, Paul-Ehrlich-lnstitute, Langen, Germany.
Further Information

Publication History

Publication Date:
08 February 2008 (online)

Abstract

Congenital factor XIII deficiency is a rare disease, but has provided valuable information on the physiological role of factor XIII and the benefit of factor XIII replacement therapy. It could be shown that not only homozygous patients but also heterozygotes are at risk for bleeding complications. Acquired factor XIII deficiency, however, is much more common, and preliminary studies suggest a lack of factor XIII to be an important feature of various diseases. In acute states and severe hemorrhages, replacement therapy with factor XIII concentrates is recommended. Recent progress in assay methods and future clinical studies should help to evaluate the therapeutic potential of factor XIII.