Affiliation:
1. Department of Haematology Sir Charles Gairdner Hospital Nedlands Australia
2. Medical School University of Western Australia Crawley Australia
3. Department of Haematology Peter MacCallum Cancer Centre and Royal Melbourne Hospital Melbourne Australia
4. Sir Peter MacCallum Department of Oncology University of Melbourne Parkville Australia
Abstract
AbstractDisease overviewMarginal zone lymphomas (MZL) are collectively the second most common type of indolent lymphoma.DiagnosisThree 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.TreatmentWe 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.
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