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[自身免疫性中性粒细胞减少症]

[Autoimmune neutropenias].

作者信息

Lamy T

机构信息

Service d'hématologie clinique CHU-Hôpital Pontchaillou 35033 Rennes.

出版信息

Rev Prat. 2001 Sep 15;51(14):1552-7.

Abstract

Primary auto-immune neutropenia (AIN) is usually described in children. Secondary AIN occurs in collagen vascular diseases such as rheumatoid arthritis and (Felty's syndrome), Gougerot-Sjogren syndrome, and systemic lupus erythematosus. Some cases of other immune cytopenia (idiopathic thrombocytopenic purpura, Evans's syndrome) or lymphoproliferative disorders (large granular lymphocyte leukemia, malignant lymphoma) may be associated with AIN. Some cases of primary AIN occur, especially in children. The diagnosis of AIN depends on the demonstration of autoantibodies directed against neutrophil-specific antigens like CD16. The availability of granulocyte-colony stimulating factor for the treatment of AIN has been a major advance. In some cases, immunosuppressive therapy using prednisone, methotrexate, cyclosporine A must be added, especially in cases of secondary AIN.

摘要

原发性自身免疫性中性粒细胞减少症(AIN)通常在儿童中出现。继发性AIN发生于胶原血管疾病,如类风湿关节炎和(费尔蒂综合征)、古热罗-舍格伦综合征以及系统性红斑狼疮。其他一些免疫性血细胞减少症(特发性血小板减少性紫癜、伊文斯综合征)或淋巴增殖性疾病(大颗粒淋巴细胞白血病、恶性淋巴瘤)的某些病例可能与AIN相关。原发性AIN的一些病例也会出现,尤其是在儿童中。AIN的诊断取决于针对中性粒细胞特异性抗原(如CD16)的自身抗体的检测。粒细胞集落刺激因子用于治疗AIN是一项重大进展。在某些情况下,必须加用泼尼松、甲氨蝶呤、环孢素A等免疫抑制疗法,尤其是在继发性AIN的病例中。

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