L'Esperance P, Brunning R, Good R A
Proc Natl Acad Sci U S A. 1973 Mar;70(3):669-72. doi: 10.1073/pnas.70.3.669.
Congenital neutropenia is a lethal disease characterized by recurrent infections beginning in the neonatal period, absence of neutrophils in the peripheral blood, eosinophilia, and monocytosis. The bone marrow shows an apparent "maturation arrest" of the neutrophil series at the promyelocyte stage. Granulocytic colonies grown in vitro in soft agar medium show normal development of eosinophilic colonies and monocyte-macrophage colonies, but defective neutrophil maturation. The abnormal colonies observed contained only myeloblasts and promyelocytes. Thus, it seems to have been possible to mimic in vitro the abnormal differentiation that is observed in vivo.
先天性中性粒细胞减少症是一种致命疾病,其特征为从新生儿期开始反复发作的感染、外周血中缺乏中性粒细胞、嗜酸性粒细胞增多和单核细胞增多。骨髓显示中性粒细胞系列在早幼粒细胞阶段明显“成熟停滞”。在软琼脂培养基中体外培养的粒细胞集落显示嗜酸性粒细胞集落和单核细胞 - 巨噬细胞集落发育正常,但中性粒细胞成熟存在缺陷。观察到的异常集落仅包含原始粒细胞和早幼粒细胞。因此,似乎有可能在体外模拟体内观察到的异常分化。