[埃文斯综合征诊断与治疗中国专家共识(2024 年)]。

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引用次数: 0

摘要

埃文斯综合征(ES)是一种罕见的自身免疫性疾病,其特征是至少存在两种自身免疫性细胞减少症(AIC),包括免疫性血小板减少症(ITP)、自身免疫性溶血性贫血(AIHA)和自身免疫性中性粒细胞减少症(AIN)。继发性 ES 占 21%-50% 的病例。ES 的特点是反复复发、严重并发症(如血栓形成和感染)和高死亡率。ES 的治疗方法多种多样,需要治疗 AIC、原发疾病及相关并发症。然而,目前仍缺乏针对 ES 的前瞻性证据和随机临床试验。为了有效规范中国的 ES 诊断和管理,中华医学会血液学分会红细胞疾病(贫血)学组制定了本共识。其目的是在国际共识的基础上,综合国内外文献,为临床实践中 ES 的诊断和管理提供有价值的指导。
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[Chinese expert consensus on the diagnosis and treatment of Evans syndrome (2024)].

Evans syndrome (ES) is a rare autoimmune disorder characterized by the presence of at least two autoimmune cytopenias (AIC), including immune thrombocytopenia (ITP), autoimmune hemolytic anemia (AIHA), and autoimmune neutropenia (AIN). Secondary ES accounts for 21%-50% of cases. ES is characterized by recurrent relapses, serious complications such as thrombosis and infection, and high mortality. The management of ES is highly heterogeneous, necessitating treatment for AIC, the primary disease, as well as associated complications. However, there remains a lack of prospective evidence, randomized clinical trials for ES. To standardize the diagnosis and management of ES in China effectively, this consensus was developed by the Red Blood Cell Diseases (Anemia) Group under the Chinese Society of Hematology within the Chinese Medical Association. It aims to provide valuable guidance for diagnosing and managing ES in clinical practice based on international consensus and comprehensive review of both domestic and international literature.

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CiteScore
0.80
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100
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