Severe bleeding complications caused by an autoantibody against the B subunit of plasma factor XIII

a novel form of acquired factor XIII deficiency

E. Ajzner, Ágota Schlammadinger, Adrienne Kerényi, Z. Bereczky, E. Katona, G. Haramura, Z. Boda, L. Muszbek

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55 Citations (Scopus)

Abstract

Acquired factor XIII (FXIII) deficiency due to autoantibody against FXIII is a very rare severe hemorrhagic diathesis. Antibodies directed against theAsubunit 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 lifethreatening bleeding in a patient with systemic lupus erythematosus. FXIII activity, FXIII-A2B 2 complex, and individual FXIII subunits were undetectable in the plasma, whereas 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, immunoglobulin, and anti- CD20 treatment resulted in the patient's recovery. FXIII levels returned to around 20% at discharge and after gradual increase the levels stabilized above 50%.

Original languageEnglish
Pages (from-to)723-725
Number of pages3
JournalBlood
Volume113
Issue number3
DOIs
Publication statusPublished - Jan 15 2009

Fingerprint

Factor XIII Deficiency
Factor XIII
Autoantibodies
Hemorrhage
Plasmas
factor XIIIb
Hemorrhagic Disorders
Passive Immunization
Plasmapheresis
Antibodies
Immunosuppressive Agents

ASJC Scopus subject areas

  • Hematology
  • Biochemistry
  • Cell Biology
  • Immunology

Cite this

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title = "Severe bleeding complications caused by an autoantibody against the B subunit of plasma factor XIII: a novel form of acquired factor XIII deficiency",
abstract = "Acquired factor XIII (FXIII) deficiency due to autoantibody against FXIII is a very rare severe hemorrhagic diathesis. Antibodies directed against theAsubunit 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 lifethreatening bleeding in a patient with systemic lupus erythematosus. FXIII activity, FXIII-A2B 2 complex, and individual FXIII subunits were undetectable in the plasma, whereas 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, immunoglobulin, and anti- CD20 treatment resulted in the patient's recovery. FXIII levels returned to around 20{\%} at discharge and after gradual increase the levels stabilized above 50{\%}.",
author = "E. Ajzner and {\'A}gota Schlammadinger and Adrienne Ker{\'e}nyi and Z. Bereczky and E. Katona and G. Haramura and Z. Boda and L. Muszbek",
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T1 - Severe bleeding complications caused by an autoantibody against the B subunit of plasma factor XIII

T2 - a novel form of acquired factor XIII deficiency

AU - Ajzner, E.

AU - Schlammadinger, Ágota

AU - Kerényi, Adrienne

AU - Bereczky, Z.

AU - Katona, E.

AU - Haramura, G.

AU - Boda, Z.

AU - Muszbek, L.

PY - 2009/1/15

Y1 - 2009/1/15

N2 - Acquired factor XIII (FXIII) deficiency due to autoantibody against FXIII is a very rare severe hemorrhagic diathesis. Antibodies directed against theAsubunit 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 lifethreatening bleeding in a patient with systemic lupus erythematosus. FXIII activity, FXIII-A2B 2 complex, and individual FXIII subunits were undetectable in the plasma, whereas 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, immunoglobulin, and anti- CD20 treatment resulted in the patient's recovery. FXIII levels returned to around 20% at discharge and after gradual increase the levels stabilized above 50%.

AB - Acquired factor XIII (FXIII) deficiency due to autoantibody against FXIII is a very rare severe hemorrhagic diathesis. Antibodies directed against theAsubunit 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 lifethreatening bleeding in a patient with systemic lupus erythematosus. FXIII activity, FXIII-A2B 2 complex, and individual FXIII subunits were undetectable in the plasma, whereas 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, immunoglobulin, and anti- CD20 treatment resulted in the patient's recovery. FXIII levels returned to around 20% at discharge and after gradual increase the levels stabilized above 50%.

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