Differential effects of granulocyte-macrophage colony-stimulating factor and granulocyte colony-stimulating factor in children with severe congenital neutropenia.
Differential effects of granulocyte-macrophage colony-stimulating factor and granulocyte colony-stimulating factor in children with severe congenital neutropenia.
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粒细胞-巨噬细胞集落刺激因子和粒细胞集落刺激因子对严重先天性中性粒细胞减少症患儿的不同作用。
作者：
K. Welte;C. Zeidler;A. Reiter;W. Muller;E. Odenwald;L. Souza;H. Riehm
严重先天性中性粒细胞减少症（SCN）是一种骨髓生成障碍，其特征是继发于早幼粒细胞水平成熟停滞的严重中性粒细胞减少症。我们用重组人粒细胞-巨噬细胞集落刺激因子（rhGM-CSF）治疗5例SCN患者42天，随后在1至3个月后，用rhG-CSF治疗142天。目的是评价这些因子引起中性粒细胞应答的安全性和能力。rhGM-CSF以3至30 μ g/kg/d的剂量给药（30至60分钟，静脉内）。在所有患者中，均观察到粒细胞绝对计数的特异性、剂量依赖性增加。然而，在4例患者中，这种增加是由于嗜酸性粒细胞增加，只有1例患者是由于绝对中性粒细胞计数（ANC）增加。随后，所有患者均接受rhG-CSF皮下注射，剂量为3至15 μ g/kg/d。与rhGM-CSF治疗相反，所有5名患者在治疗的前6周内对rhG-CSF有反应，ANC增加至1，000/μ L以上。ANC水平在维持治疗期间可以维持。在1例患者中，ANC的增加与消化链球菌引起的严重肺炎的改善相关，并且对抗生素治疗耐药。CSF治疗期间，任何患者均未发生重度细菌感染。所有患者均能耐受rhGM-CSF和rhG-CSF治疗，无严重副作用。这些结果证明了rhG-CSF在SCN患者中的有益效果。
Severe congenital neutropenia (SCN) is a disorder of myelopoiesis characterized by severe neutropenia secondary to a maturational arrest at the level of promyelocytes. We treated five patients with SCN with recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF) for 42 days and subsequently, between 1 and 3 months later, with rhG-CSF for 142 days. The objective was to evaluate the safety and ability of these factors to elicit a neutrophil response. rhGM-CSF was administered at a dose of 3 to 30 micrograms/kg/d (30 to 60 minutes, intravenously). In all patients, a specific, dose-dependent increase in the absolute granulocyte counts was observed. However, in four patients this increase was due to an increase in eosinophils, and in only one patient it was due to an increase in the absolute neutrophil counts (ANC). Subsequently, all patients received rhG-CSF at a dose of 3 to 15 micrograms/kg/d subcutaneously. In contrast to rhGM-CSF treatment, all five patients responded to rhG-CSF during the first 6 weeks of treatment with an increase in the ANC to above 1,000/microL. The level of ANC could be maintained during maintenance treatment. In one patient, the increase in ANC was associated with an improvement of a severe pneumonitis caused by Peptostreptococcus and resistant to antibiotic treatment. No severe bacterial infections occurred in any of the patients during CSF treatment. All patients tolerated rhGM-CSF and rhG-CSF treatment without severe side effects. These results demonstrate the beneficial effect of rhG-CSF in SCN patients.
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影响因子：
158.5
作者：
BONILLA, MA;GILLIO, AP;OREILLY, RJ
通讯作者：
OREILLY, RJ
重组人造血生长因子靶标的无血清培养分析：白细胞介素 3 和粒细胞/巨噬细胞集落刺激因子对早期发育阶段具有特异性。
DOI：
10.1073/pnas.85.12.4360
发表时间：
1988
影响因子：
11.1
作者：
Sonoda,Y;Yang,YC;Wong,GG;Clark,SC;Ogawa,M
通讯作者：
Ogawa,M
影响因子：
56.9
作者：
SOUZA, LM;BOONE, TC;WELTE, K
通讯作者：
WELTE, K
重组人粒细胞-巨噬细胞集落刺激因子的体外生物学特性。
影响因子：
20.3
作者：
Metcalf,D;Begley,CG;Johnson,GR;Nicola,NA;Vadas,MA;Lopez,AF;Williamson,DJ;Wong,GG;Clark,SC;Wang,EA
通讯作者：
Wang,EA
影响因子：
158.5
作者：
GABRILOVE, JL;JAKUBOWSKI, A;SOUZA, L
通讯作者：
SOUZA, L