脾边缘区淋巴瘤(SMZL)是一种少见的慢性B淋巴细胞增殖性疾病(B-CLPD),约占所有非霍奇金淋巴瘤的2%〔1〕。SMZL中位发病年龄约为65岁,发病无明显性别差异,由于和其他B-CLPD具有一定相似性因此较易误诊和漏诊。SMZL临床疾病进展缓慢,大多表现为惰性过程,10%~20%的SMZL患者在疾病过程中出现大细胞转化,进展为侵袭性大B细胞淋巴瘤〔1〕。本文主要就近年来SMZL的诊断、预后和治疗等方面的进展进行 Splenic marginal zone lymphoma (SMZL) is a rare chronic B-cell proliferative disease (B-CLPD), accounting for about 2% of all non-Hodgkin’s lymphoma [1]. The median age at onset of SMZL was approximately 65 years. There was no significant gender difference in onset. SMZL was more likely to be misdiagnosed and missed due to its similarities with other B-CLPDs. SMZL clinical disease progress is slow, most of the performance of the inert process, 10% to 20% of SMZL patients in the disease process of large cell transformation, the progress of invasive large B cell lymphoma 〔1〕. This article mainly on the SMZL in recent years, diagnosis, prognosis and treatment progress