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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Coexistance of lupus anticoagulant, factor VIII and IX inhibitor in a patient with scleroderma

演題番号 : PS-1-297

吉永 健太郎 (Kentaro Yoshinaga):1、森 直樹 (Naoki Mori):1、石森 紀子 (Noriko Ishimori):1、丁 曄 (Ye Ding):1、安並 毅 (Takeshi Yasunami):1、風間 啓至 (Hiroshi Kazama):1、志関 雅幸 (Masayuki Shiseki):1、泉二 登志子 (Toshiko Motoji):1、田中 淳司 (Junji Tanaka):1

1:Dept Hematology, Tokyo Women's Medical University, Tokyo, Japan

 

A 72-year-old woman with scleroderma was referred to the hematology section for hemostatic abnormalities. The initial coagulation test showed a prolonged bleeding time of 7.5 minutes, while prothrombin time (PT) was normal and activated partial thromboplastin time (APTT) of 100 seconds. She had neither thrombotic episodes nor spontaneous abortion. Lupus anticoagulant and anti-cardiolipin(CL) IgM were positive, but anti-CL IgG or anti-CL/β2-glycoprotein I antibody was negative. APTT was not corrected by mixing with normal plasma. Decreased factor VIII and IX activities were recognized accompanied with 7 Bethesda units (BU)/mL of factor VIII inhibitor and over 5.1 BU/mL of factor IX inhibitor. Prednisolone (PSL) and cyclophosphamide were administered for acquired hemophilia, although the therapy was not effective and discontinued due to multiple compression fracture induced by PSL. After 3 years from diagnosis, she is alive without bleeding and thrombosis, although APTT remained prolonged. Acquired hemophilia is a rare disorder, and some of patients have been observed in association with various autoimmune disorders. To our knowledge, coexistence of this combination has not yet been reported. We review literatures of previous patients with multiple inhibitors.

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