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Marginal Zone Lymphoma: 2026 Update on Diagnosis and Management - PubMed

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  • #Marginal Zone Lymphoma
  • #MZL Treatment
  • #MZL Diagnosis
  • Marginal Zone Lymphoma (MZL) is a heterogeneous group of indolent mature B-cell neoplasms with three major subtypes: nodal, splenic, and extranodal MALT.
  • Diagnosis is advanced by genomic testing, PET/CT, and emerging use of circulating tumor DNA. RECLASS classification and FIL-NF10 introduced a fourth subtype, disseminated MZL.
  • Prognosis varies by subtype and stage, with MZL IPI and FLIPI24 indices used for prognostication.
  • Frontline treatment differs for localized (radiotherapy, anti-microbial, rituximab) vs. advanced-stage (systemic immunotherapy ± chemotherapy, cBTKi-based regimens).
  • Relapsed/refractory MZL treatments include cBTKi, immunomodulators, with emerging data for antibody-drug conjugates, T-cell engagers, and CAR T-cell therapies.