Neutralizing autoantibody against factor XIII A subunit resulted in severe bleeding diathesis with a fatal outcome – characterization of the antibody

K. Pénzes, K. Rázsó, E. Katona, A. Kerényi, M. Kun, L. Muszbek

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Abstract

Essentials Autoantibody against factor XIII (FXIII) is a rare but severe acquired hemorrhagic diathesis. In an elderly patient, anti-FXIII-A antibody led to severe bleedings with fatal outcome. The neutralizing autoantibody bound to FXIII with high affinity (Ka≈109 m-1). The dominant effect of the autoantibody was the inhibition of activated FXIII. Summary: Autoantibodies may develop against the catalytic A subunit of factor XIII (FXIII-A) or the carrier B subunit (FXIII-B). Autoimmune FXIII-A deficiency was diagnosed in an elderly (75 years) patient with severe bleeding symptoms. The patient had 3% FXIII activity, and unmeasurable FXIII-A2B2 and FXIII-A antigens in the plasma, whereas, in the platelet lysate, activity and FXIII-A antigen values were normal. As revealed by western blotting, FXIII antigen was present in the plasma, but the autoantibody interfered with the immunoassays. A mixing study indicated the presence of inhibitor with a titer of 63.2 Bethesda units (BU). The patient's IgG bound to FXIII-A2B2 and to FXIII-A2 with equally high affinity (Ka in the range of 109 m−1). It exerted a multiple inhibitory effect on FXIII activation/activity (IC50: 50 μg mL−1). Immunosupressive therapy gradually decreased the autoantibody titer to 8.0 BU, but FXIII activity remained very low, and, owing to recurrent bleeding, the patient died.

Original languageEnglish
Pages (from-to)1517-1520
Number of pages4
JournalJournal of Thrombosis and Haemostasis
Volume14
Issue number8
DOIs
Publication statusPublished - Aug 1 2016

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Keywords

  • blood coagulation disorder
  • factor XIII
  • factor XIII deficiency
  • factor XIIIa
  • neutralizing antibody

ASJC Scopus subject areas

  • Hematology

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