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转化型伴原始细胞增多的难治性贫血。9例患者的临床、血液学及细胞遗传学表现

Refractory anemia with excess of blasts in transformation. Clinical, hematologic, and cytogenetic findings in nine patients.

作者信息

Smadja N, Krulik M, de Gramont A, Gonzalez-Canali G, Audebert A A, Debray J

机构信息

Clinique Médicale, Hôpital Saint-Antoine, Paris, France.

出版信息

Cancer Genet Cytogenet. 1989 Oct 1;42(1):55-65. doi: 10.1016/0165-4608(89)90008-3.

Abstract

Clinical, hematologic, and cytogenetic data of nine patients with refractory anemia with excess of blasts in transformation (RAEB-t), classified according to the French-American-British Cooperative Group for myelodysplastic syndrome (MDS), are reported. At diagnosis, eight out of nine cases, had chromosomal abnormalities and three out of nine developed acute leukemia. Karyotype studies allowed individualization of two groups of patients: five with nonrandom major karyotype abnormalities (MAKA) including hypodiploidy, chromosomes 5 and 7 involvement, at least four other abnormalities, and a poor prognosis (survival always under 3.5 months); and four patients with either normal karyotypes or minor karyotype abnormalities (MIKA) (no more than three abnormalities) and a better prognosis (survival from 14 to 38 months). Karyotype appears to be a major prognostic factor among RAEB-t.

摘要

报告了9例按照法国-美国-英国骨髓增生异常综合征(MDS)协作组分类的转化型原始细胞过多难治性贫血(RAEB-t)患者的临床、血液学和细胞遗传学数据。诊断时,9例中有8例存在染色体异常,9例中有3例发展为急性白血病。核型研究将患者分为两组:5例有非随机的主要核型异常(MAKA),包括亚二倍体、5号和7号染色体受累、至少其他4种异常,预后较差(生存期均在3.5个月以下);4例核型正常或有次要核型异常(MIKA)(不超过3种异常),预后较好(生存期为14至38个月)。核型似乎是RAEB-t的主要预后因素。

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