自身免疫性淋巴细胞增生综合征(autoimmune lymphoproliferative syndrome,ALPS)是一种以淋巴细胞大量异常存活为特征的综合征,由FAS凋亡通路失活引发,患者可逐渐发展合并为慢性淋巴细胞增生、难治性血细胞减少、自身免疫疾病,同时有较高并发恶性肿瘤的风险。ALPS最早于1967年由Canale和Smith首先发现,该病一度被认为是一种极其罕见的疾病,随着其分子病理基 Autoimmune lymphoproliferative syndrome (ALPS) is a syndrome characterized by a large number of abnormal lymphocyte survival triggered by the inactivation of the apoptotic pathway of FAS. Patients may gradually develop into chronic lymphocytic hyperplasia, Refractory cytopenias, autoimmune diseases, while at the same time have a higher risk of malignant tumors. ALPS was first discovered by Canale and Smith in 1967. It was once considered an extremely rare disease, with its molecular pathology