Recombinant human granulocyte‐colony‐stimulating factor in the treatment of patients with chronic benign granulocytopenia and congenital agranulocytosis (Kostmann's syndrome)
Recombinant human granulocyte‐colony‐stimulating factor in the treatment of patients with chronic benign granulocytopenia and congenital agranulocytosis (Kostmann's syndrome)
复制标题
重组人粒细胞集落刺激因子治疗慢性良性粒细胞减少症和先天性粒细胞缺乏症（科斯特曼综合征）患者
作者：
S. Imashuku;Masahiro Tsuchida;M. Sasaki;T. Shimokawa;H. Nakamura;T. Matsuyama;N. Taniguchi;Megumi Oda;S. Higuchi;K. Ishimoto;K. Ishimoto;M. Kobayashi;M. Kobayashi;Kazuhiro Ueda;Kazuhiro Ueda;I. Tsukimoto;I. Tsukimoto;Yoshiyuki Hanawa;Yoshiyuki Hanawa
7例慢性良性粒细胞减少症和9例先天性粒细胞缺乏症患者皮下注射重组人粒细胞集落刺激因子，起始剂量为50μg/m2/d，连续7天为一个疗程。如果没有反应，剂量增加到300μg/m2。所有慢性良性粒细胞减少症患者在用药后1-3天内以最小剂量迅速起效。相比之下，在9名先天性粒细胞缺乏症患者中，只有3人在该剂量的1-7天内有反应。4例先天性粒细胞缺乏症患者应用粒细胞集落刺激因子10 0~2 0 0μg/m2于第7~19天出现反应，其余2例无反应。粒细胞集落刺激因子的应用被证明在减少这些患者的感染发作方面也是安全和有效的。以往报道，粒细胞集落刺激因子10-30μg/kg/d治疗先天性粒细胞缺乏症有效。这些结果表明，先天性粒细胞缺乏症患者需要的重组人粒细胞集落刺激因子的剂量可能比慢性良性粒细胞减少症患者高得多，对普通剂量的重组人粒细胞集落刺激因子的反应可能有助于鉴别慢性良性粒细胞减少症和先天性粒细胞减少症。
Seven patients with chronic benign granulocytopenia and nine patients with congenital agranulocytosis, received consecutive seven‐day courses of recombinant human granulocyte‐colony stimulating factor at a starting dose of 50 μg/m2/day, subcutaneously. If there was no response the doses were increased to 300 μg/m2. All patients with chronic benign granulocytopenia responded rapidly at the minimum dose within 1–3 days after administration. By contrast, only three of the nine patients with congenital agranulocytosis responded within 1–7 days at this dose. Four patients with congenital agranulocytosis showed a response between days 7–19 at a dose of granulocyte‐colony‐stimulating factor 100‐200 μg/m2 but in the remaining two cases no response was obtained. The administration of granulocyte‐colony‐stimulating factor was shown to be safe and effective also in reducing infectious episodes in these patients. Previously it was reported that granulocyte‐colony‐stimulating factor 10–30 μg/kg/day was effective for patients with congenital agranulocytosis. These results indicate that patients with congenital agranulocytosis may require much higher doses of recombinant human granulocyte‐colony‐stimulating factor than patients with chronic benign granulocytopenia and that the response to ordinary doses of recombinant human granulocyte‐colony‐stimulating factor may be useful in differentiating between chronic benign granulocytopenia and congenital agranulocytosis.
影响因子：
158.5
作者：
BONILLA, MA;GILLIO, AP;OREILLY, RJ
通讯作者：
OREILLY, RJ
人粒细胞集落刺激因子对特发性中性粒细胞减少症患者的影响。
DOI：
10.1056/nejm198901053200107
发表时间：
1989
期刊：
The New England journal of medicine
影响因子：
--
作者：
Jakubowski,AA;Souza,L;Kelly,F;Fain,K;Budman,D;Clarkson,B;Bonilla,MA;Moore,MA;Gabrilove,J
通讯作者：
Gabrilove,J
影响因子：
56.9
作者：
SOUZA, LM;BOONE, TC;WELTE, K
通讯作者：
WELTE, K