Azuma E, Tabata N, Shibata T, Komada Y, Ito M, Atsumi S, Kawasaki Y, Ishii M, Sakurai M
Department of Pediatrics, Mie University School of Medicine, Japan.
Am J Hematol. 1990 Dec;35(4):266-8. doi: 10.1002/ajh.2830350409.
A 14-year-old boy with fatal varicella zoster virus-associated hemophagocytic syndrome (VAHS) was treated with recombinant human granulocyte colony-stimulating factor (G-CSF) based on the finding that the patient had severe neutropenia and possible bacterial superinfection. Support for the G-CSF therapy in VAHS is provided by the recent reports that G-CSF is relatively specific for the granulocyte lineage; it would not activate mature monocyte/macrophage/histiocyte lineage in VAHS, where the most striking morphologic feature is histiocytic hyperplasia with hemophagocytosis. He responded well to G-CSF with an elevation of neutrophil counts. There were no effects on other blood cells. The result indicates that G-CSF is useful to increase granulocyte production in severe neutropenia, even in the setting of fatal VAHS.
一名14岁患有致命性水痘带状疱疹病毒相关噬血细胞综合征（VAHS）的男孩，基于其存在严重中性粒细胞减少及可能的细菌重叠感染这一发现，接受了重组人粒细胞集落刺激因子（G-CSF）治疗。近期有报告指出G-CSF对粒细胞系具有相对特异性，这为VAHS中使用G-CSF治疗提供了依据；在VAHS中，最显著的形态学特征是组织细胞增生伴噬血细胞现象，G-CSF不会激活成熟的单核细胞/巨噬细胞/组织细胞系。他对G-CSF反应良好，中性粒细胞计数升高。对其他血细胞无影响。结果表明，即使在致命性VAHS的情况下，G-CSF对于严重中性粒细胞减少时增加粒细胞生成也是有用的。
