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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Lupus anticoagulant detected in a patient with acquired hemophilia A

演題番号 : PS-1-298

備後 真登 (Masato Bingo):1、一木 昭人 (Akito Ichiki):1、近澤 悠志 (Yushi Chikasawa):1、塩塚 美歌 (Mika Shiotsuka):1、丹羽 一貴 (Kazuki Niwa):1、村松 崇 (Takashi Muramatsu):1、清田 育男 (Ikuo Seita):1、四本 美保子 (Mihoko Yotsumoto):1、大瀧 学 (Manabu Otaki):1、尾形 享一 (Kyoichi Ogata):1、萩原 剛 (Takeshi Hagiwara):1、山元 泰之 (Takashi Suzuki):1、鈴木 隆史 (Takashi Suzuki):1、天野 景裕 (Kagehiro Amano):1、福武 勝幸 (Katsuyuki Fukutake):1

1:Department of Laboratory Medicine, Tokyo Medical University , Japan

 

Introduction: Acquired hemophilia A, caused by autologous antibodies to factor VIII(FVIII), is a rare but fatal bleeding disorder, while lupus anticoagulant(LA) is sometimes associated with both arterial and venous thrombosis. The coexistence of FVIII inhibitor with LA is extremely rare in the literature. We report here a case of acquired hemophilia A with detection of LA during the clinical course. Case: A 75-year-old man visited a neighboring hospital for angina and swelling of his left upper limb. CBC test showed anemia(Hb 4.4g/dl) and normal platelet count. Coagulation studies revealed normal prothrombin time and prolonged activated partial thromboplastin time(aPTT: 93.8 seconds), with decreased factor VIII activitiy(1.9%), and detection of factor VIII inhibitor(16.3 BU/ml). Therefore, he was diagnosed with acquired hemophilia A and prednisolone(1mg/kg/day) was initiated. Hemorrhagic symptoms(mainly large hematoma on his back) were resolved with disappearance of FVIII inhibitor and increased level of FVIII activity. However prolongation of aPTT was persisted(aPTT 60-70 seconds). Mixing studies of patient and normal plasma could not fully correct aPTT. LA was detected by dilute Russell's viper venom test, which was thought as a cause of aPTT prolongation. Serologic tests were negative for anti-cardiolipin antibodies and anti-beta2GPI antibodies. Conclusion: Detection of LA in a patient with acquired hemophilia A is very rare, but we need to examine it to avoid overtreatment of immunosuppressive therapy when there is a discrepancy between symptoms and coagulation studies.

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