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再生障碍性贫血的形态学及发病机制方面

[Morphological and pathogenetic aspects of aplastic anemia].

作者信息

Abdulkadyrov K M, Bessmel'tsev S S, Balashova V A, Rukavitsyn O A, Shilova E R, Rozanova O E, Bubnova L N

出版信息

Morfologiia. 1995;108(3):58-62.

PMID:8925050
Abstract

A complex study of colony-stimulating properties of cells-precursors of granulomonocytopoiesis, morphological parameters of the marrow histological structures and immunological parameters was carried out in 90 patients to specify pathogenic mechanisms of aplastic anemia (AA) development. 95% of patients appeared to have sharp decrease of the marrow colony-stimulating properties and only 5% had normal initial indexes. In most patients endostal cell number increased, while the content of reticulocytes grew higher in more than 30% of patients. Defect of the marrow stroma functioning was registered in 23% of patients. Increase of the osseous tissue volume and osteocyte number was found. Dysbalance in cellular link of immunity was demonstrated. Only temporal increase of the marrow colony-stimulating properties was marked in some patients on the background of treatment with antilymphocyte globulin, despite the clinic-hematological improvement. Multifactor pathophysiological mechanisms, the main of which is the stem cell defect lies in the base of AA pathogenesis. Hemopoietic microenvironment pathology is not the leading reason of the hemopoiesis depression but it contributes greatly in its development. Immunological alterations in patients with AA are secondary and can be partly corrected with treatment with ALG.

摘要

对90例患者进行了粒细胞单核细胞生成的细胞前体的集落刺激特性、骨髓组织结构的形态学参数和免疫学参数的综合研究,以明确再生障碍性贫血(AA)发生发展的致病机制。95%的患者骨髓集落刺激特性明显降低,只有5%的患者初始指标正常。大多数患者的骨内膜细胞数量增加,超过30%的患者网织红细胞含量升高。23%的患者出现骨髓基质功能缺陷。发现骨组织体积和骨细胞数量增加。证实免疫细胞环节失衡。尽管临床血液学有所改善,但在一些接受抗淋巴细胞球蛋白治疗的患者中,仅在治疗期间骨髓集落刺激特性出现暂时增加。多因素病理生理机制是AA发病的基础,其中主要是干细胞缺陷。造血微环境病理不是造血抑制的主要原因,但在其发展中起很大作用。AA患者的免疫改变是继发性的,用ALG治疗可部分纠正。

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