Splenic marginal zone lymphoma proposals for a revision of diagnostic, staging and therapeutic criteria

Leukemia. 2008 Mar;22(3):487-95. doi: 10.1038/sj.leu.2405068. Epub 2007 Dec 20.


Since the initial description of splenic marginal zone lymphoma (SMZL) in 1992, an increasing number of publications have dealt with multiple aspects of SMZL diagnosis, molecular pathogenesis and treatment. This process has identified multiple inconsistencies in the diagnostic criteria and lack of clear guidelines for the staging and treatment. The authors of this review have held several meetings and exchanged series of cases with the objective of agreeing on the main diagnostic, staging and therapeutic guidelines for patients with this condition. Specific working groups were created for diagnostic criteria, immunophenotype, staging and treatment. As results of this work, guidelines are proposed for diagnosis, differential diagnosis, staging, prognostic factors, treatment and response criteria. The guidelines proposed here are intended to contribute to the standardization of the diagnosis and treatment of these patients, and should facilitate the future development of clinical trials that could define more precisely predictive markers for histological progression or lack of response, and evaluate new drugs or treatments.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Antibodies, Monoclonal / therapeutic use
  • Antibodies, Monoclonal, Murine-Derived
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Antiviral Agents / therapeutic use
  • Biomarkers, Tumor / blood
  • Bone Marrow / pathology
  • Chromosome Aberrations
  • Combined Modality Therapy
  • Comorbidity
  • Diagnosis, Differential
  • Disease Management
  • Hepatitis C, Chronic / complications
  • Hepatitis C, Chronic / drug therapy
  • Humans
  • Immunophenotyping
  • Lymphoma, B-Cell, Marginal Zone* / blood
  • Lymphoma, B-Cell, Marginal Zone* / diagnosis
  • Lymphoma, B-Cell, Marginal Zone* / pathology
  • Lymphoma, B-Cell, Marginal Zone* / therapy
  • Neoplasm Staging / methods
  • Neoplasm Staging / standards
  • Practice Guidelines as Topic
  • Prognosis
  • Rituximab
  • Spleen / pathology
  • Splenectomy
  • Splenic Neoplasms* / blood
  • Splenic Neoplasms* / diagnosis
  • Splenic Neoplasms* / pathology
  • Splenic Neoplasms* / therapy


  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Murine-Derived
  • Antiviral Agents
  • Biomarkers, Tumor
  • Rituximab