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

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