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脾切除术后原发性骨髓纤维化的异基因骨髓移植

[Allogeneic bone marrow transplantation for primary myelofibrosis after splenectomy].

作者信息

Sakashita C, Akiyama H, Mori S, Ohashi K, Sakamaki H

机构信息

Department of Medicine, Tokyo Metropolitan Komagome Hospital.

出版信息

Rinsho Ketsueki. 2000 Mar;41(3):206-11.

Abstract

A 46-year-old woman was given a diagnosis of primary myelofibrosis (PMF) in 1996. Because of the progression of anemia and splenomegaly, she was scheduled for allogeneic bone marrow transplantation (BMT) from an HLA-matched sibling donor in April 1998. Cyclophosphamide and busulfan were used as the conditioning regimen. Before BMT, the patient was treated with hydroxycarbamide, which did not resolve splenomegaly. She then underwent a splenectomy, which was followed by massive portal vein thrombosis without any significant clinical outcome. After BMT, the patient obtained rapid hematologic engraftment. Moreover, the alleviation of marrow fibrosis was confirmed 4 months after BMT. We concluded that allogenic BMT can cure patients with PMF, but that the issue of splenectomy and the indications for BMT need to be evaluated further.

摘要

一名46岁女性于1996年被诊断为原发性骨髓纤维化(PMF)。由于贫血和脾肿大进展,她计划于1998年4月接受来自HLA匹配同胞供者的异基因骨髓移植(BMT)。环磷酰胺和白消安用作预处理方案。在BMT之前,患者接受了羟基脲治疗,但脾肿大未消退。然后她接受了脾切除术,术后发生了严重的门静脉血栓形成,但未产生任何明显的临床后果。BMT后,患者血液学迅速植入。此外,BMT后4个月证实骨髓纤维化有所缓解。我们得出结论,异基因BMT可以治愈PMF患者,但脾切除术问题和BMT的适应证需要进一步评估。

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