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骨髓增生异常综合征中无效造血是指什么?

What is ineffective erythropoiesis in myelodysplastic syndromes?

作者信息

Bowen D

机构信息

Department of Hematology, University of Wales College of Medicine, Health Park, Cardiff, U.K.

出版信息

Leuk Lymphoma. 1995 Jul;18(3-4):243-7. doi: 10.3109/10428199509059614.

Abstract

The anaemia of MDS is multifactorial with intramedullary ineffective erythropoiesis resulting from an imbalance between erythroid proliferation, differentiation and apoptosis in favour of initial hypercellularity with high cell death through to a lower cellularity, lower death state with the eventual evolution of the leukaemic clone in many patients. The fundamental molecular abnormality(ies) in MDS, which produce the milieu for the heterogeneous molecular insults described (e.g., oncogene mutations) remains elusive. Many questions such as why MDS red cells are macrocytic and what are the precise cellular and molecular mechanisms of ineffective erythropoiesis remain unanswered but future study of the erythroid lineage should provide molecular clues to the earliest abnormalities in the pathogenesis of MDS.

摘要

骨髓增生异常综合征(MDS)的贫血是多因素导致的,其骨髓内红细胞生成无效,这是由于红系增殖、分化和凋亡之间的失衡造成的,起初表现为细胞高度增生,伴有高细胞死亡率,直至细胞数量减少、死亡状态降低,最终许多患者会演变为白血病克隆。MDS中导致上述异质性分子损伤(如癌基因突变)环境的根本分子异常仍不清楚。许多问题,如为什么MDS患者的红细胞是大细胞性的,以及红细胞生成无效的确切细胞和分子机制是什么,仍然没有答案,但对红系谱系的未来研究应该能为MDS发病机制中最早的异常提供分子线索。

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