演題詳細

ポスター / Poster

ポスター 39 (Poster 39) :後天性血友病

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日程
2013年10月11日(金)
時間
16:50 - 17:50
会場
ポスター会場 / Poster (ロイトン札幌 3F ロイトンホールABCD)
座長・司会
森下 英理子 (Eriko Morishita):1
1:金沢大学医薬保健研究域病態検査学
 
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Three cases of acquired hemophilia A(AHA)

演題番号 : PS-1-292

釜江 剛 (Tsuyoshi Kamae):1、下村 良充 (Yoshimitsu Shimomura):1、山下 由紀子 (Yukiko Yamashita):1、三井 秀紀 (Hideki Mitsui):1、久山 純 (Jun Kuyama):1

1:Otemae Hospital

 

[Introduction] AHA is a rare disease characterized by potentially life-threatening bleeding due to autoantibodies against coagulation factor VIII. We report here 3 cases of acquired hemophilia A. [Cases] Case 1, 80-year-old man presented with large hematoma in left gluteus medius muscle. Coagulation tests confirmed the prolonged activated partial thromboplastin time (APTT), and mixing test showed the lack of APTT correction. Factor VIII activity (FVIIIa) was <1%, and factor VIII inhibitor titer was 989 BU/ml. We diagnosed as AHA. Recombinant activated factor VII (rFVIIa) and 1mg/kg/day prednisolone (PSL) were started. About 4 weeks later, APTT showed still prolonged and he became shock status because of massive bleeding in the iliopsoas muscle. Because of refractoriness for steroid therapy, we decided to treat with rituximab. After 8 courses of rituximab therapy, he achieved complete remission (CR). Case 2, 46-year-old man visited with bleeding in the muscles. Prolonged APTT was not corrected by mixing test. Coagulation tests showed FVIIIa <1% and factor VIII inhibitor titer 5 BU/ml, which confirmed the diagnosis of AHA. Because of refractoriness for steroid therapy, rituximab was started and he achieved CR. Case 3, 42-year-old woman presented with left femur pain. Coagulation tests showed APTT 65.1 seconds, FVIIIa 2%, and factor VIII inhibitor 44 BU/ml. PSL was administered and APTT improved to normal range. [Conclusion] Recombinant FVIIa is useful to stop massive bleeding episode and all three our cases achieved CR by immunosuppressive agents including rituximab.

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