6533b837fe1ef96bd12a28c7

RESEARCH PRODUCT

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

Manuela MollejoAhmet DoganFrancesc SoléEmilio IannittoCatherine ThieblemontCarlos MontalbánAndrew WotherspoonB. CoiffierM A PirisEvelyne Callet-bauchuD OscierTheodora PapadakiAlexandra Traverse-glehenVito FrancoFrançoise BergerPascale FelmanEllen D. RemsteinAntonio SalarEstella MatutesKostas Stamatopoulos

subject

Cancer Researchmedicine.medical_specialtyMEDLINElymphomaComorbiditySettore MED/08 - Anatomia PatologicaAntiviral AgentsImmunophenotypingDiagnosis DifferentialAntibodies Monoclonal Murine-DerivedBone MarrowAntineoplastic Combined Chemotherapy ProtocolsBiomarkers TumormedicineHumansCombined Modality TherapySplenic marginal zone lymphomaIntensive care medicineSplenic marginal zone lymphomaNeoplasm StagingChromosome Aberrationsbusiness.industrySplenic NeoplasmsAntibodies MonoclonalDisease ManagementLymphoma B-Cell Marginal ZoneHematologyHepatitis C ChronicPrognosismedicine.diseaseCombined Modality TherapyComorbidityLymphomaSurgeryClinical trialOncologyPractice Guidelines as TopicSplenectomyRituximabDifferential diagnosisRituximabbusinessguidelineSpleenmedicine.drug

description

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.

https://doi.org/10.1038/sj.leu.2405068