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先天性周期性中性粒细胞减少症患者的髓系祖细胞生长特征及粒细胞集落刺激因子的作用

Myeloid progenitor cell growth characteristics and effect of G-CSF in a patient with congenital cyclic neutropenia.

作者信息

Tsunogake S, Nagashima S, Maekawa R, Takano N, Kajitani H, Saito K, Enokihara H, Furusawa S, Shishido H

机构信息

Third Department of Internal Medicine, Dokkyo University School of Medicine, Tochigi, Japan.

出版信息

Int J Hematol. 1991 Jun;54(3):251-6.

PMID:1720982
Abstract

A 17-year-old male with congenital cyclic neutropenia was treated with recombinant human granulocyte colony stimulating factor (G-CSF) administered subcutaneously at 1 to 2 micrograms/kg per day. The peak and nadir counts of neutrophils and the peak counts of monocytes were significantly elevated, and the period of cycling decreased from 3 to 2 weeks. Bone marrow culture studies revealed the following abnormalities in granulocytic progenitor cells (CFU-G): a decrease in the concentrations of G-cluster forming cells, stimulated by a maximal dose of G-CSF, and a tendency of abnormally low responsive growth of the CFU-G to lower concentrations of G-CSF and GM-CSF. Our findings suggest that administration of G-CSF at relatively low doses overcomes or compensates for these abnormalities, though not completely, as fluctuation in the neutrophil counts persisted.

摘要

一名患有先天性周期性中性粒细胞减少症的17岁男性,接受了重组人粒细胞集落刺激因子(G-CSF)治疗,每天皮下注射1至2微克/千克。中性粒细胞的峰值和谷值计数以及单核细胞的峰值计数显著升高,循环周期从3周缩短至2周。骨髓培养研究显示粒细胞祖细胞(CFU-G)存在以下异常:最大剂量G-CSF刺激下G集落形成细胞浓度降低,CFU-G对较低浓度G-CSF和GM-CSF的反应性生长异常低。我们的研究结果表明,以相对低剂量施用G-CSF可克服或补偿这些异常,尽管并不完全,因为中性粒细胞计数仍有波动。

相似文献

1
Myeloid progenitor cell growth characteristics and effect of G-CSF in a patient with congenital cyclic neutropenia.先天性周期性中性粒细胞减少症患者的髓系祖细胞生长特征及粒细胞集落刺激因子的作用
Int J Hematol. 1991 Jun;54(3):251-6.
2
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Int J Hematol. 1992 Oct;56(2):113-8.
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Kinetic response of human marrow myeloid progenitor cells to in vivo treatment of patients with granulocyte colony-stimulating factor is different from the response to treatment with granulocyte-macrophage colony-stimulating factor.人类骨髓髓系祖细胞对粒细胞集落刺激因子体内治疗患者的动力学反应不同于对粒细胞-巨噬细胞集落刺激因子治疗的反应。
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Int Rev Exp Pathol. 1993;34 Pt A:215-33.

引用本文的文献

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Neutrophil disorders and their management.中性粒细胞疾病及其管理。
J Clin Pathol. 2001 Jan;54(1):7-19. doi: 10.1136/jcp.54.1.7.
2
Lenograstim. A review of its pharmacological properties and therapeutic efficacy in neutropenia and related clinical settings.来格司亭。其药理学特性及在中性粒细胞减少症和相关临床环境中的治疗效果综述。
Drugs. 1995 May;49(5):767-93. doi: 10.2165/00003495-199549050-00009.