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骨髓细胞减少的难治性骨髓增生异常综合征贫血

Refractory myelodysplastic anaemias with hypocellular bone marrow.

作者信息

Yoshida Y, Oguma S, Uchino H, Maekawa T

机构信息

Department of Medicine, Kyoto University, Japan.

出版信息

J Clin Pathol. 1988 Jul;41(7):763-7. doi: 10.1136/jcp.41.7.763.

Abstract

Thirty three patients with refractory myelodysplastic anaemias (RMDA) with marrow hypocellularity were reviewed to see whether they differed from those with normocellular or hypercellular marrows. The median age was 65 years with a male:female ratio of 26:7. There were 11 cases of refractory anaemia (RA), four of refractory anaemia with ringed sideroblasts (RARS), and 18 of refractory anaemia with excess of blasts (RAEB). All presented with peripheral cytopenias, mostly pancytopenia or bicytopenia dysplasia in one or more cell lineages, and a marrow biopsy specimen with less than normal numbers of nucleated cells for the age. Twenty four patients died, including 14 of the 16 who developed acute non-lymphocytic leukaemia (ANLL). The results suggest that patients with hypocellular RMDA have a similar prognosis to those with normocellular or hypercellular marrows at presentation.

摘要

对33例骨髓细胞减少的难治性骨髓增生异常综合征(RMDA)患者进行了回顾性研究,以观察他们是否与骨髓细胞正常或增多的患者存在差异。中位年龄为65岁,男女比例为26:7。其中有11例难治性贫血(RA),4例环形铁粒幼细胞性难治性贫血(RARS),18例原始细胞过多性难治性贫血(RAEB)。所有患者均表现为外周血细胞减少,多数为全血细胞减少或一个或多个细胞系的双血细胞减少伴发育异常,且骨髓活检标本中核细胞数量低于同年龄正常水平。24例患者死亡,其中16例发生急性非淋巴细胞白血病(ANLL)的患者中有14例死亡。结果表明,细胞减少型RMDA患者在初诊时的预后与细胞正常或增多型患者相似。

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