Gerasimova L P, Kisliak N S, Mamedova E A, Finogenova N A
Gematol Transfuziol. 1991 Mar;36(3):16-8.
The content of hemopoietic and stromal precursor-cells was studied in the bone marrow of 46 children with congenital neutropenia and of 2 children with chronic benign neutropenia. It was found that the number of GM-CFC and F-CFC in the bone marrow of patients with chronic benign neutropenia did not differ from that in the control group of normal children, and the lowering of the neutrophil number in the blood was, probably, associated with their redistribution mechanism or increased destruction in the body. Multiple defects of hemopoietic and stromal tissue were detected in children with a hereditary form of congenital neutropenia when anomalous proliferation of F-CFC and disorders in GM-CFC differentiation led to hypoplasia of granulocytic growth stem and neutropenia.
对46例先天性中性粒细胞减少症患儿及2例慢性良性中性粒细胞减少症患儿的骨髓中造血及基质前体细胞含量进行了研究。发现慢性良性中性粒细胞减少症患者骨髓中的GM-CFC和F-CFC数量与正常儿童对照组无差异,血液中中性粒细胞数量降低可能与其再分布机制或体内破坏增加有关。在遗传性先天性中性粒细胞减少症患儿中检测到造血和基质组织的多种缺陷,此时F-CFC异常增殖和GM-CFC分化紊乱导致粒细胞生长干细胞发育不全和中性粒细胞减少。