title: Clinicopathologic characteristics and treatment of marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma).
abstract: Extranodal marginal zone lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma) accounts for 7% to 8% of newly diagnosed lymphomas. Because of its association with infectious causes, such as Helicobacter pylori (HP) or Chlamydophila psittaci (CP), and autoimmune diseases, it has become the paradigm of an antigen-driven malignancy. MALT lymphoma usually displays an indolent course, and watch-and-wait strategies are justified initially in a certain percentage of patients. In patients with gastric MALT lymphoma or ocular adnexal MALT lymphoma, antibiotic therapy against HP or CP, respectively, is the first-line management of choice, resulting in lymphoma response rates from 75% to 80% after HP eradication and from 33% to 65% after antibiotic therapy for CP. In patients who have localized disease that is refractory to antibiotics, radiation is widely applied in various centers with excellent local control, whereas systemic therapies are increasingly being applied, at least in Europe, because of the potentially systemic nature of the disease. Therefore, the objective of this review is to briefly summarize the clinicopathologic characteristics of this distinct type of lymphoma along with current data on management strategies.
authors.affiliation: Programme Director for Extranodal Lymphomas, Department of Internal Medicine I, Division of Oncology, Medical University Vienna, Vienna, Austria.
authors.forename: Markus
authors.initials: M
authors.lastname: Raderer
authors.affiliation: Resident-in-Training, Department of Internal Medicine I, Division of Oncology, Medical University Vienna, Vienna, Austria.
authors.forename: Barbara
authors.initials: B
authors.lastname: Kiesewetter
authors.affiliation: Director, Unit of Lymphoid Malignancies, Division of Onco-Hematological Medicine, Department of Onco-Hematology, National Institute for Research and Treatment, San Raffaele Scientific Institute, Milano, Italy.
authors.forename: Andrés J M
authors.initials: AJ
authors.lastname: Ferreri
mesh_terms: D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000150:complications; D016481:Helicobacter Infections / Q000150:complications; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis* / Q000382:microbiology / Q000473:pathology / Q000628:therapy*; D009367:Neoplasm Staging; D011379:Prognosis; D009956:Psittacosis / Q000150:complications; D018714:Radiotherapy, Adjuvant; D012307:Risk Factors; D016896:Treatment Outcome
chemical_list: D000900:Anti-Bacterial Agents
keywords: Chlamydophila psittaci; Helicobacter pylori; antigen-driven malignancy; extranodal; non-Hodgkin lymphoma