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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Successful treatment of acquired hemophilia and pustular psoriasis during pregnancy with cyclosporin

演題番号 : PS-1-295

進藤 基博 (Motohiro Shindo):1、生田 克哉 (Katsuya Ikuta):1、土岐 康通 (Yasumichi Toki):1、畑山 真弓 (Mayumi Hatayama):1、伊藤 巧 (Satoshi Ito):1、稲村 純季 (Junki Inamura):1、鳥本 悦宏 (Yoshihiro Torimoto):2、堀 仁子 (Masako Hori):3、飯塚 一 (Hajime Iizuka):3、大石 由利子 (Yuriko Oishi):4、千石 一雄 (Kazuo Sengoku):4、高橋 伸彦 (Nobuhiko Takahashi):5、家子 正裕 (Masahiro Ieko):5、高後 裕 (Yutaka Kohgo):1

1:Division of Gastroenterology and Hematology/Oncology, Asahikawa Medical University, Japan、2:Oncology Center, Asahikawa Medical University Hospital, Japan、3:Department of Dermatology, Asahikawa Medical University, Japan、4:Department of Obstetrics and Gynecology, Asahikawa Medical University, Japan、5:Department of Internal Medicine, School of Dentistry, Health Science University of Hokkaido, Japan

 

Case:Twenties years of age woman have experienced fever and rash from her 20 weeks gestation. She had a past history of generalized pustular psoriasis in her childhood. She was admitted to our hospital at 23 weeks. Systemic administration of 30 mg prednisolone and irradiation of narrowband UVB showed only temporary effect, and she was also treated with granulocytapheresis (GCAP). GCAP was performed three times, and then she experienced bleeding tendency at the site of vascular route. APTT was determined to be 67.4 sec., and FVIII activity was found to be extremely lowered (3%). Cross-mixing test showed the existence of coagulation inhibitors, and the FVIII inhibitor titer was 2 Bethesda unit/mL, leading in the diagnosis as acquired hemophilia A. Because both generalized pustular psoriasis and acquired hemophilia should be controlled for continuation of pregnancy and delivery, the administration of 3 mg/kg cyclosporin was started. Skin lesions improved, and APTT was normalized at 32 weeks with no detectable FVIII inhibitor. She was induced to deliver a healthy infant at 39 weeks without any severe hemorrhage.
Conclusion:Prednisolone or the combination of prednisolone and cyclophosphamide were recommended for the treatment of acquired hemophilia. Generalized pustular psoriasis sometimes worsens during pregnancy, and cyclosporin is also used if other treatment is not effective. Our present case indicated that cyclosporin might be effective and safe therapeutic approach for the simultaneous control of acquired hemophilia and generalized pustular psoriasis, even in pregnancy.

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