Acquired FXIII inhibitors: a systematic review

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• 作者：Massimo Franchini (1)
  Francesco Frattini (1)
  Silvia Crestani (1)
  Carlo Bonfanti (1)
  
• 关键词：Factor XIII ; Inhibitors ; Autoantibodies ; Bleeding ; Therapy
• 刊名：Journal of Thrombosis and Thrombolysis
• 出版年：2013
• 出版时间：July 2013
• 年：2013
• 卷：36
• 期：1
• 页码：109-114
• 全文大小：216KB
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• 作者单位：Massimo Franchini (1)
  Francesco Frattini (1)
  Silvia Crestani (1)
  Carlo Bonfanti (1)
  
  1. Department of Transfusion Medicine and Hematology, Carlo Poma Hospital, Mantua, Italy
  
• ISSN：1573-742X

文摘

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.