Treatments for Marginal Zone Lymphoma.

Marginal Zone Lymphoma (MZL) is a low grade malignant B cell lymphoproliferative disorder that comprises 2% of all lymphomas. Often an enlarged spleen and villous lymphocytes are present. Many patients however may appear to have chronic lymphocytic leukemia or other lymphoproliferative disorders. Immunophenotyping can differentiate these low grade lymphoproliferative disorders. MZL frequently is CD 20 + but CD5, CD 10, and CD23−. Treatment regimens include observation, chemotherapy, splenectomy, and immunotherapy with or without chemotherapy. 20 patients over the past six have been identified with MZL based on pathologic and immunophenotypic characteristics. 6 patients have had no treatment and have had stable disease from 9 months to over 4 years. 2 patients underwent splenectomy from 3 months to 2 years from diagnosis and have stable disease. 1 patient was diagnosed at splenectomy and relapsed 5 1/2 years later and remitted with rituxin. 1 patient received weekly rituxin for 4 weeks and has been in remission for 40 months.1 patient received weekly rituxin for 4 weeks, relapsed 9 months later, and remitted with rituxin and then maintenance rituxin. 1 patient responded to weekly rituxin for 4 weeks and is receiving maintenance rituxin. 1 patient responded to weekly rituxin for 4 weeks and then had residual disease irradiated. 1 patient remains in remission after 50 months after a cervical node was irradiated. 1 patient with blastic MZL remitted with CHOP-rituxin. 2 patients progressed after rituxin and required chemotherapy. A 92 yo patient has had a partial response to rituxin. An 82 yo patient died from complications from MZL. 15/20 patients have so far avoided chemotherapy. Rituxin may be an appropriate treatment for MZL, but clinical trials will be necessary to confirm this approach.