Document Detail


Acquired factor XIII deficiency: a therapeutic challenge.
MedLine Citation:
PMID:  23306660     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Less than 60 cases of acquired factor (F)XIII deficiencies have been reported, most having distinct clinical features. To illustrate the therapeutic challenges of acquired FXIII inhibitors, we report a case of a 65-year-old patient with no previous bleeding history who suddenly developed massive haemorrhages associated to a strong and isolated FXIII inhibitor. No underlying disorder has been detected till now after three years of follow-up. Despite aggressive treatment with prednisone, rituximab, cyclophosphamide, immunoglobulin, immunoadsorption and immune tolerance his inhibitor is still present, although at low titre and with a clinical benefit since the patient has no more bleed since more than one year. Moreover the patient had a venous thromboembolic complication. After a review of the management of acquired FXIII deficiency patients and based on the management of acquired haemophilia we discuss a possible strategy for such difficult cases.
Authors:
Françoise Boehlen; Alessandro Casini; Carlo Chizzolini; Behrouz Mansouri; Hans Peter Kohler; Verena Schroeder; Guido Reber; Philippe de Moerloose
Publication Detail:
Type:  Case Reports; Journal Article; Review     Date:  2013-01-10
Journal Detail:
Title:  Thrombosis and haemostasis     Volume:  109     ISSN:  0340-6245     ISO Abbreviation:  Thromb. Haemost.     Publication Date:  2013 Mar 
Date Detail:
Created Date:  2013-03-05     Completed Date:  2013-09-18     Revised Date:  2013-09-19    
Medline Journal Info:
Nlm Unique ID:  7608063     Medline TA:  Thromb Haemost     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  479-87     Citation Subset:  IM    
Affiliation:
Division of Angiology and Haemostasis, University Hospitals of Geneva and Faculty of Medicine of Geneva, 1211 Geneva 14, Switzerland.
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MeSH Terms
Descriptor/Qualifier:
Aged
Algorithms
Antibodies, Monoclonal, Murine-Derived / therapeutic use
Cyclophosphamide / therapeutic use
Factor XIII / antagonists & inhibitors,  biosynthesis*
Factor XIII Deficiency / therapy*
Fibrinolysin / therapeutic use
Hematology / methods
Hemorrhage / chemically induced*,  therapy*
Humans
Immunoglobulins / therapeutic use
Immunosorbent Techniques
Male
Prednisone / therapeutic use
Time Factors
Venous Thromboembolism / complications,  therapy
Chemical
Reg. No./Substance:
0/Antibodies, Monoclonal, Murine-Derived; 0/Immunoglobulins; 0/rituximab; 50-18-0/Cyclophosphamide; 53-03-2/Prednisone; 9013-56-3/Factor XIII; EC 3.4.21.7/Fibrinolysin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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