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Acquired FXIII inhibitors: a systematic review.
MedLine Citation:
PMID:  23065324     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
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.
Authors:
Massimo Franchini; Francesco Frattini; Silvia Crestani; Carlo Bonfanti
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-10-14
Journal Detail:
Title:  Journal of thrombosis and thrombolysis     Volume:  -     ISSN:  1573-742X     ISO Abbreviation:  J. Thromb. Thrombolysis     Publication Date:  2012 Oct 
Date Detail:
Created Date:  2012-10-15     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9502018     Medline TA:  J Thromb Thrombolysis     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Department of Transfusion Medicine and Hematology, Carlo Poma Hospital, Mantua, Italy, massimo.franchini@aopoma.it.
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