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[红细胞生成减少症:慢性特发性或与慢性淋巴细胞白血病相关。成红细胞祖细胞培养的价值及治疗策略]

[Erythroblastopenia: chronic idiopathic or associated with chronic lymphoid leukemia. Value of cultures of erythroblastic progenitors and therapeutic strategy].

作者信息

Casadevall N, Lacombe C, Varet B

机构信息

Service d'Hématologie, Hôpital Cochin, Paris.

出版信息

Presse Med. 1993 Jun 26;22(23):1079-86.

PMID:8415459
Abstract

Fifty cases of chronic pure red cell aplasia in adults (idiopathic in 32 cases, associated with chronic lymphocytic leukaemia in 18) were followed up after studying their erythroblastic precursors in vitro. Analysis of response to immunomodulators confirmed the existence, in patients with idiopathic chronic pure red cell aplasia, of a relationship between in vitro behaviour and obtention of a remission: patients with normal differentiation of erythroblasts consistently responded to treatments, whereas treatments were usually ineffective when the erythroblasts did not differentiate in vitro. When in vitro differentiation was below normal, responses to treatments were varied. In patients with chronic lymphocytic leukaemia, the erythroblastic precursors were normal in 15/18 cases, and response to immunomodulators was observed in 13/18 cases. A second objective of this study was to determine, on a large series, the most adequate immunomodulator treatment. In idiopathic chronic pure red cell aplasia the most efficient therapy was corticosteroids and cyclophosphamide administered separately or together. The antilymphocyte serum gave disappointing results. In pure red cell aplasia associated with leukaemia cyclophosphamide was the most frequently effective drug, with corticosteroids taking second rank; however, infections were frequent. The treatments to be considered when both corticosteroids and cyclophosphamide fail are discussed.

摘要

对50例成人慢性纯红细胞再生障碍性贫血患者(32例为特发性,18例与慢性淋巴细胞白血病相关)进行了体外成红细胞前体研究后随访。对免疫调节剂反应的分析证实,在特发性慢性纯红细胞再生障碍性贫血患者中,体外行为与缓解的获得之间存在关联:成红细胞分化正常的患者对治疗始终有反应,而成红细胞在体外不分化时,治疗通常无效。当体外分化低于正常水平时,对治疗的反应各不相同。在慢性淋巴细胞白血病患者中,18例中有15例的成红细胞前体正常,18例中有13例观察到对免疫调节剂有反应。本研究的第二个目的是在大量病例中确定最适当的免疫调节剂治疗方法。在特发性慢性纯红细胞再生障碍性贫血中,最有效的治疗方法是单独或联合使用皮质类固醇和环磷酰胺。抗淋巴细胞血清的效果令人失望。在与白血病相关的纯红细胞再生障碍性贫血中,环磷酰胺是最常用的有效药物,皮质类固醇次之;然而,感染很常见。讨论了皮质类固醇和环磷酰胺均无效时应考虑的治疗方法。

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