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重组粒细胞集落刺激因子改善慢性特发性中性粒细胞减少症

[Chronic idiopathic neutropenia improved by recombinant granulocyte colony stimulating factor].

作者信息

Kojima H, Hasegawa Y, Shibuya K, Nakazawa M, Yoda Y, Abe T

机构信息

Division of Hematology, University of Tsukuba, Ibaraki, Japan.

出版信息

Rinsho Ketsueki. 1990 Feb;31(2):189-93.

PMID:1691794
Abstract

A 55-year-old man was admitted to our hospital for the evaluation of neutropenia. On physical examination, he had apthae and splenomegaly. CBC showed 1,000/microliter WBC with 5% neutrophils, and microcytic anemia consistent with iron deficiency. Bone marrow examination revealed a marked decrease of mature neutrophils, but normal percentage of immature myeloid cells. There was no morphological abnormality in the hemopoietic cells. He had no drug or family history responsible for the neutropenia. Anti-neutrophil auto-antibody was negative. Hence, a diagnosis of chronic idiopathic neutropenia (CIN) was made. He developed frequent episodes of infection such as balanitis, peri-anal infection, gingivitis, and pharyngitis. He was treated with steroid pulse therapy, anabolic hormone, and high dose gamma-globulin infusion, but no significant improvement occurred. Then, recombinant granulocyte-colony stimulating factor (rG-CSF) was started. The neutrophil count was normalized by the 7th day of 5 micrograms/kg/day rG-CSF administration. The administration of G-CSF was discontinued after a 14-day course. Thereafter, the neutrophil count remained at near normal level (approximately 1,500/microliter) and there have been no episodes of infection in the last 5 months. However this cannot be explained simply by the direct effect of rG-CSF on the myeloid precursors; rather, it suggests some unknown effect of G-CSF on the bone marrow microenvironment regulating myeloid hemopoiesis. We consider this to be a rare case of CIN with frequent episodes of infection, which was successfully treated with G-CSF.

摘要

一名55岁男性因中性粒细胞减少症入院接受评估。体格检查发现他有口腔溃疡和脾肿大。全血细胞计数显示白细胞为1000/微升,中性粒细胞占5%,且存在与缺铁相符的小细胞性贫血。骨髓检查显示成熟中性粒细胞显著减少,但未成熟髓细胞百分比正常。造血细胞无形态学异常。他没有导致中性粒细胞减少症的药物或家族史。抗中性粒细胞自身抗体为阴性。因此,诊断为慢性特发性中性粒细胞减少症(CIN)。他频繁发生感染,如龟头炎、肛周感染、牙龈炎和咽炎。他接受了类固醇脉冲疗法、同化激素和大剂量丙种球蛋白输注治疗,但无明显改善。随后,开始使用重组粒细胞集落刺激因子(rG-CSF)。在每天给予5微克/千克rG-CSF的第7天,中性粒细胞计数恢复正常。在14天疗程后停止给予G-CSF。此后,中性粒细胞计数维持在接近正常水平(约1500/微升),且在过去5个月中未发生感染。然而,这不能简单地用rG-CSF对髓系祖细胞的直接作用来解释;相反,这提示G-CSF对调节髓系造血的骨髓微环境有一些未知作用。我们认为这是一例罕见的伴有频繁感染发作的CIN病例,经G-CSF治疗成功。

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