Autrel-Moignet Aline, Lamy Thierry
CHU de Rennes, service d'hématologie clinique, Rennes 35043, France.
CHU de Rennes, service d'hématologie clinique, Rennes 35043, France; Université Rennes 1, Rennes 35043, France.
Presse Med. 2014 Apr;43(4 Pt 2):e105-18. doi: 10.1016/j.lpm.2014.02.007. Epub 2014 Mar 27.
Autoimmune neutropenia (AIN) is a rare entity caused by antibodies directed against neutrophil-specific antigens. It includes primary and secondary autoimmune neutropenia. Acute autoimmune neutropenia can be related to drug-induced mechanism or viral infections. Chronic autoimmune neutropenias occur in the context of autoimmune diseases, hematological malignancies, such as large granular lymphocyte leukemia, primary immune deficiency syndromes or solid tumors. The therapeutic management depends on the etiology. Granulocyte growth factor is the main therapeutic option, raising the question of their long-term utilization safety. Corticosteroids or immunosuppressive therapy are indicated in infection-related AIN or in case of symptomatic autoimmune disease or LGL leukemia.
自身免疫性中性粒细胞减少症（AIN）是一种由针对中性粒细胞特异性抗原的抗体引起的罕见病症。它包括原发性和继发性自身免疫性中性粒细胞减少症。急性自身免疫性中性粒细胞减少症可能与药物诱导机制或病毒感染有关。慢性自身免疫性中性粒细胞减少症发生于自身免疫性疾病、血液系统恶性肿瘤（如大颗粒淋巴细胞白血病）、原发性免疫缺陷综合征或实体瘤的背景下。治疗管理取决于病因。粒细胞生长因子是主要的治疗选择，这引发了其长期使用安全性的问题。皮质类固醇或免疫抑制疗法适用于感染相关的AIN或有症状的自身免疫性疾病或大颗粒淋巴细胞白血病的情况。
