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异基因骨髓移植成功治疗原发性骨髓纤维化

[Primary myelofibrosis successfully treated with allogeneic bone marrow transplantation].

作者信息

Iwata N, Inoue N, Tamura A, Miyazaki E, Fujimori Y, Okamoto T, Takemoto Y, Kosaki M, Kanamaru A, Kakishita E

机构信息

2nd Department of Internal Medicine, Hyogo College of Medicine.

出版信息

Rinsho Ketsueki. 1992 Nov;33(11):1703-7.

PMID:1469787
Abstract

A 31-year-old man with primary myelofibrosis initially received low dose Ara C. Splenomegaly decreased but pancytopenia continued. Allogeneic bone marrow transplantation from his sister was then performed. Busulfan and cyclophosphamide were used as a preconditioning regimen, which included neither irradiation nor splenectomy. As the bone marrow was hypoplastic after transplantation, G-CSF was given. It was useful for systemic infection. After transplantation, leukoerythroblastosis and tear drop poikilocytosis disappeared in peripheral blood. Finally, bone marrow fibrosis disappeared and hemopoiesis to normal limits recovered 17 months later. These results demonstrate that bone marrow transplantation is effective for primary myelofibrosis for which there is no otherwise curative therapy.

摘要

一名31岁的原发性骨髓纤维化男性患者最初接受了小剂量阿糖胞苷治疗。脾肿大有所减轻,但全血细胞减少仍持续存在。随后进行了来自其妹妹的异基因骨髓移植。白消安和环磷酰胺被用作预处理方案,该方案既未包括放疗也未包括脾切除术。由于移植后骨髓发育不全,给予了粒细胞集落刺激因子(G-CSF)。它对全身感染有效。移植后,外周血中的幼稚粒-幼红细胞增多症和泪滴状异形红细胞消失。最终,骨髓纤维化消失,17个月后造血恢复至正常范围。这些结果表明,骨髓移植对于原发性骨髓纤维化有效,而原发性骨髓纤维化尚无其他治愈性治疗方法。

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