Cheah Chan Y, Seymour John F
Department of Haematology, Sir Charles Gairdner Hospital, Nedlands, Australia.
Medical School, University of Western Australia, Crawley, Australia.
Am J Hematol. 2023 Oct;98(10):1645-1657. doi: 10.1002/ajh.27058. Epub 2023 Aug 21.
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.
边缘区淋巴瘤（MZL）是第二常见的惰性淋巴瘤类型。
MZL可分为三种亚型：脾型、结外型和结内型。通过对受累的结内或结外部位进行活检确诊，活检显示克隆性B细胞浸润，免疫表型最常见为CD5和CD10阴性。部分病例会出现IgM副蛋白，但MYD88 L256P突变的发生率低于华氏巨球蛋白血症。预后 已开发出多种预后模型，包括MALT-IPI和MZL-IPI。后者广泛适用于MZL各亚型，并将血清乳酸脱氢酶升高、贫血、淋巴细胞减少、血小板减少以及结内或播散型亚型作为独立的预后预测指标。
我们将参考化学免疫疗法、放射疗法以及复发/难治性疾病的新兴治疗方法（如BTK抑制剂），讨论针对早期和晚期疾病的建议治疗方法。
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