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 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-A(2)B(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%.

journal_name

Blood

journal_title

Blood

authors

Ajzner E,Schlammadinger A,Kerényi A,Bereczky Z,Katona E,Haramura G,Boda Z,Muszbek L

doi

10.1182/blood-2008-09-179333

subject

Has Abstract

pub_date

2009-01-15 00:00:00

pages

723-5

issue

3

eissn

0006-4971

issn

1528-0020

pii

blood-2008-09-179333

journal_volume

113

pub_type

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