Prognosis in marginal zone lymphoma: a comprehensive review

Abstract

Marginal zone lymphomas (MZLs) represent a distinct subset of indolent B-cell lymphomas, including extranodal, nodal, and splenic variants. Despite shared histopathological and immunophenotypic features, their clinical presentation varies significantly based on the site of origin and genetic abnormalities. The heterogeneity of the disease is reflected in its management, particularly in therapeutic strategies and prognosis determination.

Several prognostic tools have been developed to refine risk assessment in MZL subtypes, including the Mucosa-Associated Lymphoid Tissue International Prognostic Index (MALT-IPI), the Haemoglobin Platelets Lactate Dehydrogenase Lymphadenopathy (HPLL) score and its simplified version. Recently, the Marginal Zone Lymphoma International Prognostic Index (MZL-IPI) has emerged as a comprehensive model integrating data from all MZL subtypes and incorporating rigorously validated clinical parameters.

This review aims to synthesize current knowledge on MZL prognosis, critically assess existing prognostic models, and highlight the potential clinical impact of identifying risk stratification.