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

Leukemia volume 22, pages 487–495 (2008)Cite this article

Abstract

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.

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Acknowledgements

Other active participants in the preparatory meetings for this manuscript, and members of the Splenic Marginal Zone Lymphoma group, were Lucile Baseggio, Martine French, Jose A Martínez-Climent, Jesus M Hernandez-Rivas, Dolores Caballero, Zadie Davies, Alison Morilla, David Gonzalez, Marta Salido, Wolfram Klapper and Reiner Siebert. Preparatory work has been partially funded by grants from the Ministerio de Sanidad y Consumo (G03/179, PI051623, PI052800), the Ministerio de Ciencia y Tecnología (SAF2005-00221, SAF2004-04286) and Fundacion la Caixa, Spain.

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Authors and Affiliations

  1. Section of Haemato-Oncology, Institute of Cancer Research, London, UK

    E Matutes & A Wotherspoon

  2. Department of Haematology, Royal Bournemouth Hospital, Bournemouth, UK

    D Oscier

  3. Servicio de Medicina Interna, Hospital Ramon y Cajal, Madrid, Spain

    C Montalban

  4. Department of Pathology and Hematology, Centre Hospitalier Lyon Sud, Lyon, France

    F Berger, E Callet-Bauchu, P Felman, A Traverse-Glehen & B Coiffier

  5. Department of Pathology, Mayo Clinic, Rochester, MN, USA

    A Dogan & E D Remstein

  6. Department of Pathology and Hematology, University of Palermo, Palermo, Italy

    V Franco & E Iannitto

  7. Department of Pathology, Hospital Virgen de la Salud, Toledo, Spain

    M Mollejo

  8. Department of Pathology, Evangelismos Hospital, Athens, Greece

    T Papadaki

  9. Department of Pathology, Hospital del Mar, Barcelona, Spain

    A Salar & F Solé

  10. Department of Hematology, G Papanicolau Hospital, Thessaloniki, Greece

    K Stamatopoulos

  11. Department of Hematology, Hopital Saint Louis, Paris, France

    C Thieblemont

  12. Programa de Patologia Molecular, Centro Nacional de Investigaciones Oncologicas, Madrid, Spain

    M A Piris

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  1. E Matutes
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  10. M Mollejo
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Correspondence to M A Piris.

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Matutes, E., Oscier, D., Montalban, C. et al. Splenic marginal zone lymphoma proposals for a revision of diagnostic, staging and therapeutic criteria. Leukemia 22, 487–495 (2008). https://doi.org/10.1038/sj.leu.2405068

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