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
Bloodjournal_title
Bloodauthors
Ajzner E,Schlammadinger A,Kerényi A,Bereczky Z,Katona E,Haramura G,Boda Z,Muszbek Ldoi
10.1182/blood-2008-09-179333subject
Has Abstractpub_date
2009-01-15 00:00:00pages
723-5issue
3eissn
0006-4971issn
1528-0020pii
blood-2008-09-179333journal_volume
113pub_type
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