[再生障碍性贫血发病42年后转化为伴有der(1;7)的骨髓增生异常综合征]

[Myelodysplastic syndrome with der (1;7) transformed from aplastic anemia 42 years after onset].

作者信息

Kanemura Nobuhiro, Tsurumi Hisashi, Hara Takeshi, Yamada Toshiki, Moriwaki Hisataka

机构信息

First Department of Internal Medicine, Gifu University Graduate School of Medicine.

出版信息

Rinsho Ketsueki. 2005 May;46(5):372-4.

PMID:16444972

Abstract

A 51-year-old man had been diagnosed with severe aplastic anemia (AA) at the age of 9 years. He was treated with occasional transfusions for the next 35 years. Following initiation of cyclosporin A (CsA), the patient became transfusion-independent at 44-years-old. Anemia improved after cessation of CsA and no therapy was required for the next 5 years. However, severe pancytopenia suddenly developed at the age of 51. Bone marrow aspiration revealed myelodysplastic syndrome (MDS) with der (1;7). The ineffectiveness of CsA for MDS led to resumed dependence on transfusion. This case suggests that the appearance of MDS clones might have contributed to transient hematological improvement. Bone marrow aspiration should be considered in patients with AA if unexpected hematological improvement appears.

摘要

一名51岁男性在9岁时被诊断为严重再生障碍性贫血（AA）。在接下来的35年里，他偶尔接受输血治疗。开始使用环孢素A（CsA）后，患者在44岁时不再依赖输血。停用CsA后贫血得到改善，接下来的5年无需治疗。然而，51岁时突然出现严重全血细胞减少。骨髓穿刺显示为伴有der(1;7)的骨髓增生异常综合征（MDS）。CsA对MDS无效导致再次依赖输血。该病例提示MDS克隆的出现可能促成了短暂的血液学改善。如果AA患者出现意外的血液学改善，应考虑进行骨髓穿刺。