State-of-the-art therapeutics: marginal-zone lymphoma

J Clin Oncol. 2005 Sep 10;23(26):6415-20. doi: 10.1200/JCO.2005.05.018.

Abstract

Marginal-zone lymphomas comprise the mucosa-associated lymphoid tissue (MALT) type (extranodal marginal-zone lymphoma [EMZL]), the nodal marginal zone B-cell lymphoma (NMZL) and the splenic MZL (SMZL). EMZL is relatively common, whereas the remaining two entities are relatively rare disorders. EMZL, especially in its gastric localization, is the most studied MZL, and there are many data both on the underlying genetic lesions and on the role of infectious agents. These data have determined unique approach among all other lymphoma subtypes: the possibility of treating a subset of patients with antibiotics alone as first line of treatment. Indeed, there is compelling evidence that histologic regressions can be achieved in most gastric MALT lymphomas by eradicating Helicobacter pylori infection. However, molecular follow-up studies showed the persistence of the malignant clone in half of the cases in histologic remission after antibiotic treatment and transient, either histologic or molecular, relapses have been reported, too. Hence, a careful long-term follow-up is mandatory after antibiotic treatment. Radiotherapy, chemotherapy, anti-CD20 monoclonal antibodies are effective alternative therapies. The precise role of surgical resection should be redefined in view of the encouraging results of conservative approaches. Differently from EMZL, both SMLZ and NMZL often present with disseminated disease at diagnosis. The therapeutic approach comprises splenectomy, for SMZL, and chemotherapy, but with no consensus about the best treatment. This review addresses the current knowledge on the clinical features and therapeutic approaches for the individual MZLs.

Publication types

  • Comparative Study
  • Review

MeSH terms

  • Anti-Bacterial Agents / therapeutic use*
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Combined Modality Therapy
  • Female
  • Helicobacter Infections / diagnosis
  • Helicobacter Infections / drug therapy*
  • Helicobacter Infections / epidemiology
  • Helicobacter pylori / drug effects
  • Helicobacter pylori / isolation & purification
  • Humans
  • Immunotherapy / methods*
  • Lymphoma, B-Cell, Marginal Zone / epidemiology
  • Lymphoma, B-Cell, Marginal Zone / pathology*
  • Lymphoma, B-Cell, Marginal Zone / therapy*
  • Male
  • Prognosis
  • Radiotherapy, Adjuvant
  • Randomized Controlled Trials as Topic
  • Risk Assessment
  • Severity of Illness Index
  • Survival Analysis
  • Treatment Outcome

Substances

  • Anti-Bacterial Agents