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成人获得性再生障碍性贫血。I. 40例回顾性分析:影响预后的单一因素

Acquired aplastic anaemia in adults. I. A retrospective analysis of 40 cases: single factors influencing the prognosis.

作者信息

Haak H L, Hartgrink-Groeneveld C A, Eernisse J G, Speck B, van Rood J J

出版信息

Acta Haematol. 1977;58(5):257-77. doi: 10.1159/000207837.

Abstract

In a retrospective analysis of 40 aplastic anaemia patients, an attempt was made to determine prognostic parameters permitting discrimination between short survivors (less than 6 months) and long-term survivors (greater than 6 months). Short survival proved to be significantly associated with a persistently low reticulocyte index and progressive neutropenia. Other factors such as bone marrow cellularity, HBF level, aetiology, or presenting signs were not indicative. Chromosomal aberrations were found in one third of the cases examined, but none had developed into a leukaemic stage after an observation period of 2--5 years. None of the patients with cytogenetic aberrations showed complete restoration of haematopoiesis, in contrast to several of the patients without chromosomal abnormalities. In this series, drug-associated aplastic anaemia was found more often in the short-survival group. These clinical findings indicate that the term 'aplastic anaemia' covers a heterogenous group of disorders.

摘要

在对40例再生障碍性贫血患者进行的回顾性分析中,试图确定能够区分短期存活者(少于6个月)和长期存活者(多于6个月)的预后参数。结果表明,短期存活与持续低网织红细胞指数和进行性中性粒细胞减少显著相关。其他因素,如骨髓细胞密度、胎儿血红蛋白水平、病因或首发症状,并无指示性意义。在所检查的病例中,三分之一发现有染色体畸变,但经过2至5年的观察期后,无一例发展为白血病阶段。与一些无染色体异常的患者相反,有细胞遗传学畸变的患者均未显示造血功能完全恢复。在该系列中,药物相关性再生障碍性贫血在短期存活组中更为常见。这些临床发现表明,“再生障碍性贫血”一词涵盖了一组异质性疾病。

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