title: Splenic marginal zone lymphoma: current knowledge and future directions.
abstract: Splenic marginal zone lymphoma (SMZL), along with extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) and nodal marginal zone lymphoma (NMZL), share a common origin from the "marginal zone." However, these three entities display different clinical characteristics, reflecting probable biological variations according to the organ and cellular origin. Within the past decade, new data have been reported regarding pathogenic mechanisms as well as therapeutic advances. Clinically, SMZL presents as an indolent and disseminated disease at diagnosis, with a specific clinical presentation that includes predominantly splenomegaly, and in half of patients, autoimmune manifestations. Establishing the diagnosis may be difficult, especially distinguishing SMZL from other low-grade lymphomas, such as small B-cell lymphomas; however, recent findings have contributed to a better characterization of the disease, and the criteria for diagnosis have been improved. Therapeutic approaches consist of splenectomy or immunochemotherapy, but there is no consensus regarding the best treatment, except when SMZL is associated with hepatitis C virus infection. In this article, we review the current knowledge on the biological findings, clinical features, and therapeutic approaches for SMZL.
authors.affiliation: Hôpital Saint-Louis, Paris, France. catherine.thieblemont@sls.aphp.fr
authors.forename: Catherine
authors.initials: C
authors.lastname: Thieblemont
authors.forename: Frederic
authors.initials: F
authors.lastname: Davi
authors.forename: Maria-Elena
authors.initials: ME
authors.lastname: Noguera
authors.forename: Josette
authors.initials: J
authors.lastname: Brière
authors.forename: Francesco
authors.initials: F
authors.lastname: Bertoni
authors.forename: Emanuele
authors.initials: E
authors.lastname: Zucca
authors.forename: Alexandra
authors.initials: A
authors.lastname: Traverse-Glehen
authors.forename: Pascale
authors.initials: P
authors.lastname: Felman
authors.forename: Françoise
authors.initials: F
authors.lastname: Berger
authors.forename: Gilles
authors.initials: G
authors.lastname: Salles
authors.forename: Bertrand
authors.initials: B
authors.lastname: Coiffier
mesh_terms: D006526:Hepatitis C / Q000150:complications; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis / Q000209:etiology / Q000473:pathology / Q000628:therapy*; D009154:Mutation; D011379:Prognosis; D013160:Splenic Neoplasms / Q000175:diagnosis / Q000209:etiology / Q000473:pathology / Q000628:therapy*
chemical_list: D007143:Immunoglobulin Heavy Chains