title: Splenic marginal zone lymphoma.
abstract: 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.
authors.affiliation: Istituto di Anatomia e Istologia Patologica and Divisione di Ematologia con TMO, University of Palermo, Palermo, Italy. vfranco@unipa.it
authors.forename: Vito
authors.initials: V
authors.lastname: Franco
authors.forename: Ada Maria
authors.initials: AM
authors.lastname: Florena
authors.forename: Emilio
authors.initials: E
authors.lastname: Iannitto
mesh_terms: D000818:Animals; D020732:Cytogenetic Analysis; D003937:Diagnosis, Differential; D006801:Humans; D016130:Immunophenotyping; D016393:Lymphoma, B-Cell / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D008228:Lymphoma, Non-Hodgkin / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D013160:Splenic Neoplasms / Q000235:genetics / Q000473:pathology* / Q000628:therapy