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难治性贫血(RA)及伴环状铁粒幼细胞的难治性贫血中某些临床、形态学和细胞遗传学表现的预后意义

Prognostic significance of some clinical, morphological and cytogenetic findings in refractory anaemia (RA) and RA with sideroblasts.

作者信息

Kerndrup G, Pedersen B, Ellegaard J, Hokland P

出版信息

Blut. 1986 Jan;52(1):35-43. doi: 10.1007/BF00320140.

Abstract

In 27 patients initially diagnosed as refractory anaemia (RA) or RA with sideroblasts (RA-S) according to the FAB-classification a number of clinical, morphological and cytogenetic parameters were correlated for prognostic significance. From these correlations it emerged that severe cytopenia is centrally positioned with regard to clinical course in RA and RA-S. Positive correlations were found to initial diagnosis, clonal cytogenetic abnormalities, progression to RA with an excess of blasts (RAEB) or acute myeloid leukaemia (AML), the percentage of bone marrow blast cells and prolonged half life for radioactively labeled iron. The degree of peripheral blood granulocytopenia, alone, was correlated to bone marrow hypoplasia. Moreover, the frequency of abnormal karyotypes was inversely correlated to bone marrow cellularity and proportional to the frequency of bone marrow blast cells. From these relationships it may be proposed that chromosome abnormalities are associated with prolonged blast cell generation times and inhibition of blast cell maturation resulting in reduced marrow cellularity and blast cell accumulation, and, in the peripheral blood, falling percentages of neutrophil granulocytes. With the blast cell accumulation the bone marrow cellularity again becomes hyperplastic and the preleukaemic condition is transformed into RAEB or AML.

摘要

根据FAB分类法,对27例最初诊断为难治性贫血(RA)或伴有环形铁粒幼细胞的难治性贫血(RA-S)患者的一些临床、形态学和细胞遗传学参数进行了相关性分析以评估预后意义。从这些相关性分析中发现,严重血细胞减少在RA和RA-S的临床病程中处于核心地位。发现其与初始诊断、克隆性细胞遗传学异常、进展为伴有过多原始细胞的RA(RAEB)或急性髓系白血病(AML)、骨髓原始细胞百分比以及放射性标记铁的半衰期延长呈正相关。仅外周血粒细胞减少程度与骨髓发育不全相关。此外,异常核型的频率与骨髓细胞数量呈负相关,与骨髓原始细胞频率成正比。从这些关系可以推测,染色体异常与原始细胞生成时间延长和原始细胞成熟抑制有关,导致骨髓细胞数量减少和原始细胞积聚,在外周血中,中性粒细胞百分比下降。随着原始细胞积聚,骨髓细胞数量再次增生,白血病前期状态转变为RAEB或AML。

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