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[中性粒细胞选择性发育不全:自身免疫起源]

[Selective aplasia of neutrophils: auto-immune origin].

作者信息

Roth P, Pugin P, Dottrens A, Odavic R, Deubelbeiss K A

出版信息

Schweiz Med Wochenschr. 1979 Sep 29;109(37):1397-8.

PMID:314666
Abstract

Autoimmunity is a recognized factor in pure red cell aplasia and in some cases of aplastic anemia, but not in agranulocytosis. The case reported here demonstrated that pure neutrophilic aplasia of autoimmune origin may exist in man. A 75-year-old male is described who presented with complete agranulocytosis and absence of neutrophilic precursors in the bone marrow without thrombocytopenia or anemia. After 6 weeks, remission was induced by immunosuppressive treatment and 22 months later the patient is doing well without therapy. The absence of known cases of agranulocytosis, a good response to immunosuppressive therapy, and the demonstrated fact that the patient's peripheral blood mononuclear cells inhibit the growth of granulocytic colonies of normal human marrow in vitro allow the conclusion that this patient had pure neutropenic aplasia, probably due to autoimmune phenomena.

摘要

自身免疫是纯红细胞再生障碍性贫血以及某些再生障碍性贫血病例中的一个公认因素,但在粒细胞缺乏症中并非如此。本文报道的病例表明,人类可能存在自身免疫性起源的纯中性粒细胞再生障碍。描述了一名75岁男性,其表现为完全性粒细胞缺乏,骨髓中无中性粒细胞前体,无血小板减少或贫血。6周后,免疫抑制治疗诱导缓解,22个月后患者未经治疗情况良好。缺乏已知的粒细胞缺乏症病例、对免疫抑制治疗的良好反应以及患者外周血单个核细胞在体外抑制正常人骨髓粒细胞集落生长这一已证实的事实,使得我们得出结论,该患者患有纯中性粒细胞再生障碍,可能是由于自身免疫现象所致。

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