边缘区淋巴瘤（MZL）是集体中第二常见的慢性淋巴瘤类型。MZL又可分为三个亚型：脾脏型、非淋巴结型和淋巴结型。诊断通过对受累淋巴结或非淋巴结部位进行活检，显示具有CD5和CD10阴性免疫表型最常见的B细胞克隆浸润来确立。有些病例会出现IgM帕拉蛋白，但MYD88 L256P突变在华氏大球蛋白血症中出现的频率较低。预后几个预后模型已被开发出来，包括MALT-IPI和MZL-IPI。后者在各个MZL亚型中普遍适用，并将血清LDH升高、贫血、淋巴细胞减少、血小板减少和淋巴结或广泛播散亚型作为独立的预后预测因子加以综合。我们将讨论对早期和晚期疾病的治疗建议，包括化学免疫疗法、放射治疗以及在复发/难治疾病中出现的新兴治疗如BTK抑制剂等。©2023 The Authors. American Journal of Hematology由Wiley Periodicals LLC出版。

Marginal zone lymphomas (MZL) are collectively the second most common type of indolent lymphoma.Three subtypes of MZL are recognized: splenic, extranodal, and nodal. The diagnosis is secured following biopsy of an involved nodal or extranodal site demonstrating a clonal B-cell infiltrate with CD5 and CD10 negative immunophenotype most common. Some cases will features IgM paraprotein, but MYD88 L256P mutations are less frequent than in Waldenstrom macroglobulinemia. Prognostication Several prognostic models have been developed, including the MALT-IPI and the MZL-IPI. The latter is broadly applicable across MZL subtypes and incorporates elevated serum LDH, anemia, lymphopenia, thrombocytopenia and nodal or disseminated subtypes as independent predictors of outcome.We discuss suggested approach to therapy for both early and advanced-stage disease, with reference to chemo-immunotherapy, radiotherapy, and emerging treatments in relapsed/refractory disease such as BTK inhibitors.© 2023 The Authors. American Journal of Hematology published by Wiley Periodicals LLC.