Abstract: Autoinflammatory diseases （AIDs） are a group of genetic diseases that have attracted high attention in recent years， and their main characteristics are systemic inflammatory responses caused by innate immune dysregulation， and unlike autoimmune diseases， they usually lack autoantibodies or antigen-specific T cells. AIDs are more common than juvenile-onset illness and can affect multiple systems throughout the body， with the hematologic system being the most common site of AIDs involvement， and they share many common clinical features with hematologic neoplasms， such as anemia， lymphadenopathy， and/or splenomegaly. While hematopoietic cells help create and propagate a protective inflammatory response to infection or injury， excessive inflammation can lead to many diseases of the blood， bone marrow， and lymphatic system . This article focuses on several common hematologic manifestations of AIDs in order to raise awareness among haematologists about this type of disease and to avoid misdiagnosis or mistreatment.

Key words: autoinflammatory diseases, autoinflammation, hematology, clinical manifestations, differential diagnosis

摘要： 自身炎症性疾病（AIDs）是近年来备受关注的一组遗传性疾病，主要特征是在缺乏病原体、循环自身抗体或自身反应性T细胞的情况下，先天性免疫应答增强。这些疾病的临床特征是无菌性多器官炎症的反复发作，可累及全身多个系统，血液系统是AIDs受累的最常见系统，它们与血液系统肿瘤有许多共同的临床特征，如贫血、淋巴结肿大和（或）脾脏肿大。虽然造血细胞有助于产生和传播对感染或损伤的保护性炎症反应，但是过度炎症可导致血液、骨髓和淋巴系统的多种疾病。文章主要探讨AIDs在血液学方面的几种典型表现，旨在提高对这类疾病的认识，减少误诊或漏诊。

关键词: 自身炎症性疾病, 自身炎症, 血液学, 临床表现, 鉴别诊断