Acquired FXIII inhibitors: a systematic review

J Thromb Thrombolysis. 2013 Jul;36(1):109-14. doi: 10.1007/s11239-012-0818-3.


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.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Autoantibodies* / blood
  • Autoantibodies* / immunology
  • Autoimmune Diseases / blood
  • Autoimmune Diseases / complications
  • Autoimmune Diseases / immunology
  • Autoimmune Diseases / therapy
  • Blood Coagulation Factor Inhibitors* / blood
  • Blood Coagulation Factor Inhibitors* / immunology
  • Factor XIII Deficiency* / blood
  • Factor XIII Deficiency* / etiology
  • Factor XIII Deficiency* / immunology
  • Factor XIII Deficiency* / therapy
  • Factor XIII* / antagonists & inhibitors
  • Factor XIII* / immunology
  • Factor XIII* / metabolism
  • Fibrin / immunology
  • Fibrin / metabolism
  • Humans
  • Lymphoproliferative Disorders / blood
  • Lymphoproliferative Disorders / complications
  • Lymphoproliferative Disorders / immunology
  • Lymphoproliferative Disorders / therapy


  • Autoantibodies
  • Blood Coagulation Factor Inhibitors
  • Fibrin
  • Factor XIII