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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Five cases of acquired hemophilia A treated with immunosuppressive therapy

演題番号 : PS-1-291

門平 靖子 (Yasuko Kadohira):1、中川 紀温 (Noriharu Nakagawa):1、井美 達也 (Tatsuya Imi):1、大畑 欣也 (Kinya Ohata):1、小谷 岳春 (Takeharu Kotani):1、林 朋恵 (Tomoe Hayashi):1、森下 英理子 (Eriko Morishita):1、朝倉 英策 (Hidesaku Asakura):1、中尾 眞二 (Shinji Nakao):1

1:Cellular Transplantation Biology, Kanazawa University, Japan

 

[Introduction]Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies against coagulation factor VIII. We report five cases of acquired hemophilia A treated with immunosuppressive therapy.[Case] Median age onset was 63 years old (30-85). Four cases were male and one was female. Three cases had underlying diseases: gastric cancer, bile duct cancer and psoriasis vulgaris. Two cases had no underlying diseases. Factor VIII activity was 1-5% in four cases, >5% in two . FVIII inhibitor titer was 1.0-154.0 BU/ml. Subcutaneous bleeding was observed in all cases, and intramuscular bleeding was observed in three cases. We used the bypassing agents for hemostasis in four cases. All patients were treated with prednisolone as immunosuppressive therapy. Cyclophosphamide was added to two of these patients. Rituximab was added to another patient. Both additive immunosuppressive drugs were administerd in short period. Inhibitors disappeared in all cases. Median time to disappearance of inhibitor was 7.4 weeks (3-12). One case showed a relapse after several months. Three cases had pneumonia during treatment.[Discussion] The patients with acquired hemophilia were elderly persons except one case. Such patients as having underlying diseases were difficult to treat, and it took a long time to the disappearance of inhibitor. The patients with malignant diseases were more pronounced. Long-term immunosuppressive therapy increases the risk of complications such as infection. It is important to select appropriate therapies and manage underlying diseases.

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