Komiyama A, Yamazaki M, Yoda S, Saitoh H, Morosawa H, Akabane T
Am J Hematol. 1981 Sep;11(2):175-82. doi: 10.1002/ajh.2830110209.
In order to obtain further knowledge of chronic neutropenia of childhood, we studied nine neutropenic infants six to ten months of age by in vitro techniques, including bone marrow culture, electron microscopy, and chemotaxis assay. Eight of the nine patients had a benign clinical course and the bone marrow aspirates showed a reduced number of segmented neutrophils. The ninth patient had a moderately severe course and the bone marrow showed maturation arrest at the promyelocyte stage. Bone marrow cultures demonstrated that the in vitro neutrophil colony formation and production of colony-stimulating activity were normal in all of the eight patients studied. Neutrophils from one of the nine patients had ultrastructural abnormalities such as decrease in number of primary and secondary granules and the presence of myelin figures in primary granules. Neutrophil chemotaxis was defective in three of the nine patients. All of the six patients in whom the neutrophil colony formation in agar, the ultrastructure of neutrophils, and neutrophil chemotaxis were normal recovered from the neutropenia between 11 and 30 months of age. These in vitro parameters appear to be useful for evaluating chronic neutropenia of childhood.
为了进一步了解儿童慢性中性粒细胞减少症，我们采用体外技术对9名6至10个月大的中性粒细胞减少婴儿进行了研究，这些技术包括骨髓培养、电子显微镜检查和趋化性测定。9名患者中有8名临床病程良性，骨髓穿刺显示分叶核中性粒细胞数量减少。第9名患者病程中度严重，骨髓显示早幼粒细胞阶段成熟停滞。骨髓培养表明，在研究的8名患者中，所有患者的体外中性粒细胞集落形成和集落刺激活性的产生均正常。9名患者中有1名患者的中性粒细胞存在超微结构异常，如初级和次级颗粒数量减少以及初级颗粒中出现髓鞘样结构。9名患者中有3名患者的中性粒细胞趋化性存在缺陷。6名中性粒细胞在琼脂中的集落形成、中性粒细胞超微结构和中性粒细胞趋化性均正常的患者在11至30个月大时从中性粒细胞减少症中康复。这些体外参数似乎有助于评估儿童慢性中性粒细胞减少症。
