6533b82bfe1ef96bd128d88e

RESEARCH PRODUCT

Splenic marginal zone lymphoma.

Emilio IannittoAda Maria FlorenaVito Franco

subject

BendamustinePathologymedicine.medical_specialtyLymphoma B-CellLymphocytosismedicine.medical_treatmentImmunologySplenectomyBiochemistryImmunophenotypingDiagnosis DifferentialImmunophenotypingMedicineAnimalsHumansSplenic marginal zone lymphomabusiness.industryLymphoma Non-HodgkinSplenic NeoplasmsCell BiologyHematologymedicine.diseaseLymphomaTumor progressionCytogenetic AnalysisRituximabmedicine.symptombusinessmedicine.drug

description

Splenic marginal zone lymphoma (SMZL) is a specific low-grade small B-cell lymphoma that is incorporated in the World Health Organization classification. Characteristic features are splenomegaly, moderate lymphocytosis with villous morphology, intrasinusoidal pattern of involvement of various organs, especially bone marrow, and relative indolent course. Tumor progression with increase of blastic forms and aggressive behavior are observed in a minority of patients. Molecular and cytogenetic studies have shown heterogeneous results probably because of the lack of standardized diagnostic criteria. To date, no definitive therapy has been established. Therapeutic options include treatment abstention, splenectomy, splenic irradiation, and chemotherapy.

10.1182/blood-2002-07-2216https://pubmed.ncbi.nlm.nih.gov/12446449