ACQUIRED FACTOR VIII INHIBITORS IN NON-HEMOPHILIC PATIENTS: CLINICAL EXPERIENCE OF 16 CASES
Abstract number: P-W-220
Zheng1 C.C., Wu1 J.S.
1Department of Hematology, Anhui Provincial Hospital, Hefei City, China
How-to-cite Zheng CC, Wu JS. ACQUIRED FACTOR VIII INHIBITORS IN NON-HEMOPHILIC PATIENTS: CLINICAL EXPERIENCE OF 16 CASES. J Thromb Haemost 2007; 5 Supplement 2: P-W-220
Introduction: Acquired factor VIII inhibitors are antibodies that inactivate function of a clotting factor FVIII developed in non-hemophilia (Acquired hemophilia, AH) patients. Sixteen AH patients since 1995 to 2006 in our hospital were reviewed.
Methods: AH was diagnosed on the basis of a recent onset of a bleeding history and a reduced FVIII level caused by an inhibitor to FVIII demonstrated in a Bethesda assay.
Results: There were 9 female and 7 male patients. The median age was 50.5years (21- 75 years). Fourteen patients had subcutaneous bleeding, and 9 patients had soft tissue hematoma. Eight patients had autoimmune diseases such as systemic lupus erythematosus, rheumatoid arthritis, and Sjogren syndrome. Others included gastric carcinoma and postoperation of ectopic pregnancy. No underlying disorder was observed in 6 patients. Mean factor VIII level was 5.10 IU/dl (0.12- 26.98IU/dl), mean titer of inhibitor was 12 BU (0.85-77 BU). Substitute treatments included prothrombin complex concentrate (30-50 U/kg every 12-24h,), and high-dose human factor VIII concentrate (50-100 U/kg every 12h). According to the titer of inhibitor, we divided these patients into two groups: low responders(<5 Bethesda units, n=9) and high responders (>5 Bethesda Unit, n=7). Six low responders and all high responders were treated with immunosuppressive therapy such as prednisone and cyclophosphamide, 3 high responders also received high- dose intravenous immunoglobulin(IV IG, 0.4g/kg/d, 5 days). All low responders obtained complete remission(CR). Only 3 high responders obtained CR, 2 patients obtained partial remission, 2 patients discontinued immunosuppressive treatment, then died due to severely uncontrollable hemorrhage.
Conclusions: AH patients had highly variable clinical courses and responses to immunosuppressive therapy, elimination of inhibitors may be delayed specifically in patients with high inhibitor titres.
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Journal of Thrombosis and Haemostasis 2007; Volume 5, Supplement 2: abstract number
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