Marginal zone lymphoma: 2023 update on diagnosis and management
Author(s)
Cheah, CY; Seymour, JF;
Details
Publication Year 2023-10,Volume 98,Issue #10,Page 1645-1657
Journal Title
American Journal of Hematology
Publication Type
Review
Abstract
DISEASE OVERVIEW: Marginal zone lymphomas (MZL) are collectively the second most common type of indolent lymphoma. DIAGNOSIS: 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. TREATMENT: 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.
Publisher
Wiley
Keywords
Humans; *Lymphoma, B-Cell, Marginal Zone/diagnosis/genetics/therapy; B-Lymphocytes; Adaptor Proteins, Signal Transducing; Biopsy; Immunophenotyping
Department(s)
Clinical Haematology
PubMed ID
37605344
Open Access at Publisher's Site
https://doi.org/10.1002/ajh.27058
Terms of Use/Rights Notice
Refer to copyright notice on published article.


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