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Blood, 15 January 2009, Vol. 113, No. 3, pp. 723-725. Prepublished online as a Blood First Edition Paper on October 27, 2008; DOI 10.1182/blood-2008-09-179333. This Article Full Text (PDF) All Versions of this Article: blood-2008-09-179333v1 113/3/723    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Ajzner, E. Articles by Muszbek, L. Search for Related Content PubMed PubMed Citation Articles by Ajzner, E. Articles by Muszbek, L. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted September 17, 2008 Accepted October 17, 2008 Severe bleeding complications caused by an autoantibody against the B subunit of plasma factor XIII; a novel form of acquired factor XIII deficiency Eva Ajzner, Agota Schlammadinger, Adrienne Kerenyi, Zsuzsanna Bereczky, Eva Katona, Gizella Haramura, Zoltan Boda, and Laszlo Muszbek* Clinical Research Center, University of Debrecen, Medical and Health Science Center, Debrecen, Hungary 2nd Department of Medicine, Hemostasis Division, University of Debrecen, Medical and Health Science Center, Debrecen, Hungary Department of Clinical Biochemisty and Molecular Pathology, University of Debrecen, Medical and Health Science Center, Debrecen, Hungary Thrombosis, Haemostasis and Vascular Biology Research Group of the Hungarian Academy of Sciences, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary * Corresponding author; email: muszbek{at}med.unideb.hu. Acquired factor XIII (FXIII) deficiency due to autoantibody against FXIII is a very rare severe hemorrhagic diathesis. Antibodies directed against the A subunit of FXIII, which interfere with different functions of FXIII, have been described. Here, for the first time, we report an autoantibody against the B subunit of FXIII (FXIII-B) that caused life-threatening bleeding in a patient with systemic lupus erythematosus. FXIII activity, FXIII-A2B2 complex and individual FXIII subunits were undetectable in the plasma, while platelet FXIII activity and antigen were normal. Neither FXIII activation nor its activity was inhibited by the antibody, which bound to structural epitope(s) on both free and complexed FXIII-B. The autoantibody highly accelerated the elimination of FXIII from the circulation. FXIII supplementation combined with immunosuppressive therapy, plasmapheresis, immunglobulin and anti-CD20 treatment resulted in the patient's recovery. FXIII levels returned to around 20% at discharge and after gradual increase it stabilized above 50%. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: C. Jambor, V. Reul, T. W. Schnider, P. Degiacomi, H. Metzner, and W. C. Korte In Vitro Inhibition of Factor XIII Retards Clot Formation, Reduces Clot Firmness, and Increases Fibrinolytic Effects in Whole Blood Anesth. Analg., October 1, 2009; 109(4): 1023 - 1028. [Abstract] [Full Text] [PDF]

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