1. ¹Section of Haemato-Oncology, Institute of Cancer Research, London, UK
2. ²Department of Haematology, Royal Bournemouth Hospital, Bournemouth, UK
3. ³Servicio de Medicina Interna, Hospital Ramon y Cajal, Madrid, Spain
4. ⁴Department of Pathology and Hematology, Centre Hospitalier Lyon Sud, Lyon, France
5. ⁵Department of Pathology, Mayo Clinic, Rochester, MN, USA
6. ⁶Department of Pathology and Hematology, University of Palermo, Palermo, Italy
7. ⁷Department of Pathology, Hospital Virgen de la Salud, Toledo, Spain
8. ⁸Department of Pathology, Evangelismos Hospital, Athens, Greece
9. ⁹Department of Pathology, Hospital del Mar, Barcelona, Spain
10. ¹⁰Department of Hematology, G Papanicolau Hospital, Thessaloniki, Greece
11. ¹¹Department of Hematology, Hopital Saint Louis, Paris, France
12. ¹²Programa de Patologia Molecular, Centro Nacional de Investigaciones Oncologicas, Madrid, Spain

Correspondence: Dr MA Piris, Molecular Pathology Program, Centro Nacional de Investigaciones Oncologicas, Melchor Frenandez Almagro 3, Madrid 28029, Spain. E-mail: mapiris@cnio.es

Received 28 September 2007; Revised 15 November 2007; Accepted 16 November 2007; Published online 20 December 2007.

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.