Yasui K, Yamazaki M, Miyagawa Y, Komiyama A, Akabane T
Am J Hematol. 1987 May;25(1):67-75. doi: 10.1002/ajh.2830250107.
Childhood chronic neutropenia with decreased numbers of chemotactic factor receptors as well as defective chemotaxis was first demonstrated in an 8-month-old girl. Chemotactic factor receptors on neutrophils were assayed using tritiated N-formyl-methionyl-leucyl-phenylalanine (3H-FMLP). The patient's neutrophils had decreased numbers of the receptors: numbers of the receptors were 20,000 (less than 3 SD) as compared with those of control cells of 52,000 +/- 6,000 (mean +/- SD) (n = 10). The neutropenia disappeared spontaneously by 28 months of age parallel with the improvement of chemotaxis and increase in numbers of chemotactic factor receptors. These results demonstrate a transient decrease of neutrophil chemotactic factor receptors as one of the pathophysiological bases of a transient defect of neutrophil chemotaxis in this disorder.
一名8个月大的女孩首次被证实患有儿童慢性中性粒细胞减少症，其趋化因子受体数量减少且趋化功能存在缺陷。使用氚标记的N-甲酰甲硫氨酰亮氨酰苯丙氨酸（3H-FMLP）对中性粒细胞上的趋化因子受体进行检测。该患者中性粒细胞的受体数量减少：受体数量为20,000（低于3个标准差），而对照细胞的数量为52,000±6,000（平均值±标准差）（n = 10）。中性粒细胞减少症在28个月大时自发消失，同时趋化功能得到改善，趋化因子受体数量增加。这些结果表明，中性粒细胞趋化因子受体的短暂减少是该疾病中性粒细胞趋化功能短暂缺陷的病理生理基础之一。
