Journal of Thrombosis and Thrombolysis

, Volume 36, Issue 1, pp 109–114

Acquired FXIII inhibitors: a systematic review

Authors

    • Department of Transfusion Medicine and HematologyCarlo Poma Hospital
  • Francesco Frattini
    • Department of Transfusion Medicine and HematologyCarlo Poma Hospital
  • Silvia Crestani
    • Department of Transfusion Medicine and HematologyCarlo Poma Hospital
  • Carlo Bonfanti
    • Department of Transfusion Medicine and HematologyCarlo Poma Hospital
Article

DOI: 10.1007/s11239-012-0818-3

Cite this article as:
Franchini, M., Frattini, F., Crestani, S. et al. J Thromb Thrombolysis (2013) 36: 109. doi:10.1007/s11239-012-0818-3

Abstract

Coagulation factor XIII (FXIII) is a protein that promotes fibrin stabilization by forming multiple covalent cross-links between fibrin monomers. Beside congenital FXIII deficiency, due to FXIII gene mutations, severe acquired FXIII deficiency has been described in association with autoantibodies against coagulation FXIII. These inhibitors, which occurs very rarely but may cause life-threatening bleeding complications, may arise spontaneously or in association with autoimmune and lymphoproliferative disorders or medications. The management of patients with acquired FXIII inhibitors is very demanding and treatment regimens must be focused on eradication of the inhibitor and to increase the plasma FXIII levels. In this systematic review, we analyse all the published case-reports on anti-FXIII autoantibodies focusing on the clinical features and treatment modalities of this acquired hemorrhagic condition.

Keywords

Factor XIII Inhibitors Autoantibodies Bleeding Therapy

Copyright information

© Springer Science+Business Media New York 2012