Non-MALT marginal zone lymphomas: Diagnostic and therapeutic challenges

Haema 2013; 4(2):151-160

by Christina Kalpadakis1, Gerassimos A. Pangalis2, Maria K. Angelopoulou3, Theodoros P. Vassilakopoulos3

1Hematological Clinic University General Hospital of Ηerakleion, University Medical School, Crete,
2Department of Hematology, Athens Medical Center, Psychikon Branch, Athens,
3Department of Haematology, National and Kapodistrian University of Athens, Laiko General Hospital, Goudi, Athens, Greece

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Splenic and nodal marginal zone lymphomas (SMZL and NMZL) represent low-grade lymphomas which are considered as distinct entities in the WHO classification. Despite similarities in morphology and immunophenotype of lymphomatous cells, they differ in many aspects e.g. clinical presentation, clinical course, genomic aberrations, immunogenetic signatures and response to therapy. Important advances have been recorded recently in both understanding the pathogenesis of these diseases and also in improving their treatment. Immunogenetic analysis of SMZL provided strong evidence for antigen involvement in lymphomagenesis. Significant progress has also been made in the management of SMZL. Recent studies showed that the monoclonal antibody rituximab is very effective in SMZL with minimal toxicity, rephaps rendering splenectomy no longer necessary. The diagnosis of NMZL presents many difficulties and is a challenge for pathologists. Differential diagnosis from other low-grade lymphomas can be cumbersome and collaboration between haematologists and pathologists is mandatory for establishment of the diagnosis. Therapy is influenced by the experience from other low-grade lymphomas, mainly follicular lymphomas. The addition of rituximab in the therapeutic armamentarium improved significantly the outcome of patients with NMZL.