Young G A, Iland H J, Deveridge S F, Forrest P R, Vincent P C
Blut. 1984 Mar;48(3):153-9. doi: 10.1007/BF00320338.
A 19-year old girl with severe cyclical neutropenia associated with life-threatening infection and who responded dramatically to the administration of oral prednisolone is described. During reduction and eventual cessation of steroid therapy normal or near normal neutrophil counts have been maintained, and there has been parallel improvement in clinical well-being. Prior to therapy and at a time of peak blood neutrophil count low numbers of granulocyte-macrophage progenitor cells (CFU-C) were found in the patient's bone marrow, and her lymphocytes co-cultured with normal marrow failed to show the inhibitory effect normally seen with normal lymphocytes. The findings in this patient are compared with those in the two other cases where cyclical neutropenia has been shown to respond to steroids.
本文描述了一名19岁患有严重周期性中性粒细胞减少症并伴有危及生命感染的女孩,该女孩对口服泼尼松龙治疗反应显著。在逐渐减少并最终停止类固醇治疗期间,中性粒细胞计数维持在正常或接近正常水平,临床状况也同步改善。治疗前及血中性粒细胞计数峰值时,在患者骨髓中发现少量粒细胞-巨噬细胞祖细胞(CFU-C),且其淋巴细胞与正常骨髓共同培养时未显示出正常淋巴细胞通常所见的抑制作用。将该患者的研究结果与另外两例已证明对类固醇有反应的周期性中性粒细胞减少症病例的结果进行了比较。