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成人自身免疫性中性粒细胞减少症。

Autoimmune neutropenia in adults.

机构信息

Department of Hematology and Medical Oncology, Winship Cancer Institute, Emory University School of Medicine, Atlanta, GA 30322, USA.

出版信息

Autoimmun Rev. 2009 Sep;9(1):62-6. doi: 10.1016/j.autrev.2009.03.006. Epub 2009 Mar 14.

Abstract

Autoimmune neutropenias (AIN) in adults are a heterogeneous group of diseases with clinical manifestations varying from being asymptomatic to having infectious complications with considerable morbidity and mortality. They are characterized by autoantibodies directed against neutrophils, resulting in destruction of neutrophils. AIN can be divided into two forms. In primary AIN, neutropenia is usually the sole hematologic abnormality and it is more common in children. Secondary AIN, which is more prevalent in adults, is associated with underlying autoimmune diseases, malignancies, infections, particularly viral, neurological diseases or drug exposure. This article is an overview of these conditions with emphasis on secondary AIN; it also discusses the available serological methods for antibody detection and recent therapeutic developments including colony stimulating factors, rituximab and Campath-1H.

摘要

成人自身免疫性中性粒细胞减少症(AIN)是一组异质性疾病,临床表现从无症状到伴有严重感染并发症和死亡率不等。它们的特征是针对中性粒细胞的自身抗体导致中性粒细胞破坏。AIN 可分为两种形式。在原发性 AIN 中,中性粒细胞减少症通常是唯一的血液学异常,并且更常见于儿童。继发性 AIN 更常见于成人,与自身免疫性疾病、恶性肿瘤、感染(特别是病毒)、神经疾病或药物暴露有关。本文概述了这些疾病,重点介绍继发性 AIN;还讨论了用于抗体检测的现有血清学方法以及包括集落刺激因子、利妥昔单抗和 Campath-1H 在内的最新治疗进展。

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