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[伴有显著嗜酸性粒细胞增多和嗜碱性粒细胞增多的骨髓增生异常综合征]

[Myelodysplastic syndrome associated with marked eosinophilia and basophilia].

作者信息

Tanabe J, Sasaki S, Tamura T, Okamoto R, Sugamura R, Fujita H, Fukawa H, Kanamori H, Matsuzaki M, Mohri H

机构信息

First Department of Internal Medicine, Yokohama City University.

出版信息

Rinsho Ketsueki. 1992 Feb;33(2):189-93.

PMID:1635167
Abstract

Myelodysplastic syndrome (refractory anemia with excess of blasts; RAEB) with marked basophilia and eosinophilia is described. An 82-year-old male was admitted to our hospital because of severe normocytic normochromic anemia (Hb 5.6 g/dl). The white cell count was 9,200/microliters with marked basophilia (34.5%) and eosinophilia (19.5%). The bone marrow aspiration also revealed both basophilia and eosinophilia, with blast contents of 9%. Diagnosis of RAEB was established. Although the treatment with red cell transfusion and ubenimex (Bastatin) was started, anemia was not improved. A karyotype of the bone marrow cells from this patient showed 47, XY, +8, i (17q), which has been observed as additional chromosomal abnormalities in blastic crisis of chronic myelogenous leukemia. The diagnosis of CML was not compatible with this case, because Ph1 chromosome and bcr gene rearrangement were negative. It is concluded that eosinophilia and basophilia might be derived from clonal abnormalities associated with MDS.

摘要

描述了伴有明显嗜碱性粒细胞增多和嗜酸性粒细胞增多的骨髓增生异常综合征(难治性贫血伴原始细胞增多;RAEB)。一名82岁男性因严重正细胞正色素性贫血(血红蛋白5.6 g/dl)入住我院。白细胞计数为9200/微升,伴有明显的嗜碱性粒细胞增多(34.5%)和嗜酸性粒细胞增多(19.5%)。骨髓穿刺也显示嗜碱性粒细胞增多和嗜酸性粒细胞增多,原始细胞含量为9%。确诊为RAEB。尽管开始了红细胞输注和乌苯美司(抑氨肽酶B)治疗,但贫血并未改善。该患者骨髓细胞的核型显示为47,XY,+8,i(17q),这在慢性粒细胞白血病的急变期已被观察到为额外的染色体异常。慢性粒细胞白血病的诊断与该病例不符,因为Ph1染色体和bcr基因重排均为阴性。结论是嗜酸性粒细胞增多和嗜碱性粒细胞增多可能源于与骨髓增生异常综合征相关的克隆异常。

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