title: Splenic marginal zone lymphoma: disease features and management.
abstract: Splenic marginal zone lymphoma (SMZL) is a lymphoma recognized as a distinct entity in the WHO classification of the lymphoid tumors. SMZL probably results from the expansion of a marginal zone B-cell driven by persistent antigen stimulation. SMZL is clinically and biologically heterogeneous. The SMZL Working Group has published guidelines for the diagnosis, workup and treatment of SMZL. There are no standard criteria to initiate treatment. A policy of watch and wait in asymptomatic patients is recommended. In symptomatic patients, data from retrospective studies suggest that rituximab with or without chemotherapy is the best strategy for SMZL. It is uncertain which is the optimal type of chemotherapy and whether patients may benefit from splenectomy prior chemoimmunotherapy. In the future, we may see progress with agents targeting known molecular lesions in SMZL.
authors.affiliation: Haematopathology Unit, Hospital Clinic . Barcelona University, Villarroel, 170, 08036 Barcelona, Spain +34 663 109 312 +34 932 275 717 estella.matutes.juan@gmail.com.
authors.forename: Estella
authors.initials: E
authors.lastname: Matutes
mesh_terms: D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002986:Clinical Trials as Topic; D006801:Humans; D016898:Interferon-alpha / Q000627:therapeutic use; D016393:Lymphoma, B-Cell / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D011379:Prognosis; D011687:Purines / Q000737:chemistry / Q000627:therapeutic use; D051883:Receptor, Notch2 / Q000037:antagonists & inhibitors / Q000378:metabolism; D012254:Ribavirin / Q000627:therapeutic use; D000069283:Rituximab; D013160:Splenic Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D016898:Interferon-alpha; C497764:NOTCH2 protein, human; D011687:Purines; D051883:Receptor, Notch2; D012254:Ribavirin; D000069283:Rituximab; C030985:purine