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[骨髓增生异常综合征中的染色体异常]

[Chromosome abnormalities in myelodysplastic syndrome].

作者信息

Fujita K, Mori H, Niikura H, Terada H, Shinohara T

机构信息

Department of Blood Center, Fujigaoka Hospital, Showa University, Yokohama.

出版信息

Rinsho Byori. 1990 Aug;38(8):911-6.

PMID:2232253
Abstract

We performed chromosomal analysis in 18 patients with myelodysplastic syndrome (MDS). According to the French-American-British (FAB) cooperative study group and Research Group of Japanese Ministry of Welfare Classification, our cases with MDS were classified into four subtypes as follows; refractory anemia [RA], 6 cases; refractory anemia with excess of blasts [RAEB], 4; chronic myelomonocytic leukemia [CMML], 3; refractory anemia with excess of blasts in transformation [RAEB-T], 4; and refractory cytopenia [RC], 1. Thirteen patients (72%) had chromosomal abnormalities and frequently observed chromosomal abnormalities were trisomy 8, -7/7q-, 20q-, trisomy 1q and 5q-. The mean survival were as follows; RA: 22.5 months, RAEB: 13.2 months, CMML: 15 months, RAEB-T: 5.5 months. Progression to overt leukemia occurred in 5 patients (27.7%): 1 of four patients with RAEB, 1 of three patients with CMML and 3 of four patients with RAEB-T. In conclusion, chromosomal abnormalities were most frequently observed in the patient with RAEB-T who had shortest survival time among the patients with MDS. On the other hand, chromosomal abnormalities were less frequently observed in the patients with RA and they showed relatively better prognosis than the other types of MDS.

摘要

我们对18例骨髓增生异常综合征(MDS)患者进行了染色体分析。根据法美英(FAB)协作研究组和日本厚生省研究组的分类,我们的MDS病例分为以下四种亚型:难治性贫血[RA],6例;伴有原始细胞增多的难治性贫血[RAEB],4例;慢性粒-单核细胞白血病[CMML],3例;转变中的伴有原始细胞增多的难治性贫血[RAEB-T],4例;难治性血细胞减少[RC],1例。13例患者(72%)存在染色体异常,常见的染色体异常为8三体、-7/7q-、20q-、1q三体和5q-。平均生存期如下:RA:22.5个月,RAEB:13.2个月,CMML:15个月,RAEB-T:5.5个月。5例患者(27.7%)进展为明显白血病:4例RAEB患者中的1例,3例CMML患者中的1例,4例RAEB-T患者中的3例。总之,染色体异常在RAEB-T患者中最常见,该组患者在MDS患者中生存期最短。另一方面,RA患者染色体异常较少见,且其预后相对优于其他类型的MDS。

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