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抗体介导的单纯中性粒细胞缺乏症、复发性重症肌无力及既往胸腺瘤:病例报告与文献综述

Antibody-mediated pure neutrophil aplasia, recurrent myasthenia gravis and previous thymoma: case report and literature review.

作者信息

Mathieson P W, O'Neill J H, Durrant S T, Henderson S J, Green P J, Newsom-Davis J

机构信息

Department of Clinical Neurology, National Hospital for Nervous Diseases, Queen Square, London.

出版信息

Q J Med. 1990 Jan;74(273):57-61.

PMID:2326435
Abstract

We describe a case of neutrophil aplasia in a woman with recurrent myasthenia gravis and a past history of thymoma. Bone marrow showed virtually absent granulopoiesis but normal erythropoiesis and megakaryopoiesis. Bone marrow cultures showed no growth of granulocyte/mononuclear cell progenitors (CFU-GM). She was treated with immunosuppression including azathioprine, and her neutrophil count returned to normal. Serum before treatment, and also an IgG fraction thereof, inhibited CFU-GM growth both in autologous 'remission' marrow and in allogeneic marrow. She remains in complete remission 36 months after starting azathioprine. This association is extremely rare, and was formerly associated with a grim prognosis.

摘要

我们描述了一例患有复发性重症肌无力且既往有胸腺瘤病史的女性发生中性粒细胞发育不全的病例。骨髓显示粒细胞生成几乎缺失,但红细胞生成和巨核细胞生成正常。骨髓培养显示粒细胞/单核细胞祖细胞(CFU-GM)无生长。她接受了包括硫唑嘌呤在内的免疫抑制治疗,中性粒细胞计数恢复正常。治疗前的血清及其IgG组分均抑制自体“缓解期”骨髓和异体骨髓中CFU-GM的生长。开始使用硫唑嘌呤36个月后,她仍处于完全缓解状态。这种关联极为罕见,以前预后很差。

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