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真性红细胞增多症继发骨髓纤维化采用减低强度干细胞移植治疗成功。

Successful treatment with reduced-intensity stem cell transplantation for secondary myelofibrosis following polycythemia vera.

作者信息

TAHARA Kenichi, YOKOHAMA Akihiko, HANDA Hiroshi, SAITOH Takayuki, UCHIUMI Hideki, SEKIGAMI Tomomi, TOYAMA Kohtaro, MAWATARI Momoko, OSAKI Yohei, MATSUSHIMA Takafumi, KARASAWA Masamitsu, MURAKAMI Hirokazu, TSUKAMOTO Norifumi, NOJIMA Yoshihisa

机构信息

Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine.

出版信息

Rinsho Ketsueki. 2009 Nov;50(11):1630-4.

PMID:20009439
Abstract

Ten years after being diagnosed with polycythemia vera, a 55-year-old woman required frequent blood transfusion due to secondary myelofibrosis. She underwent reduced-intensity stem cell transplantation (RIST) from an HLA-identical sibling donor. Since mixed chimerae were identified in the peripheral blood at day 35, cyclosporine was withdrawn. At day 73, she developed acute graft-versus-host disease of the liver, while simultaneous resolution of splenomegaly occurred and complete donor chimerism in the peripheral blood was achieved. Frequent red blood cell transfusion was required until day 300 after transplantation. Thus, RIST for an older patient with secondary myelofibrosis was successful without severe treatment-related morbidity. This case suggests that RIST could be an effective treatment modality for secondary myelofibrosis.

摘要

一名55岁女性在被诊断为真性红细胞增多症10年后,因继发性骨髓纤维化需要频繁输血。她接受了来自HLA配型相同的同胞供者的减低强度干细胞移植(RIST)。由于在第35天外周血中检测到混合嵌合体,停用了环孢素。在第73天,她发生了急性肝移植物抗宿主病,同时脾肿大消退,外周血实现了完全供者嵌合。移植后300天内需要频繁输注红细胞。因此,对于老年继发性骨髓纤维化患者,RIST治疗成功,且无严重的治疗相关并发症。该病例提示RIST可能是继发性骨髓纤维化的一种有效治疗方式。

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