Nongastric Marginal Zone B-Cell Lymphoma: Analysis of 247 Cases - Clinical Presentation and Treatment Outcomes of Nongastric Marginal Zone B-Cell Lymphoma.

Purpose: Nongastric marginal zone B-cell lymphoma (NG-MZL) is a relatively uncommon indolent lymphoma. We report a retrospective analysis of 247 patients with .NG-MZL, presenting their clinical features and therapeutic outcomes.

Methods: From 1990 to 2005, a total of 247 patients with histologically confirmed NG-MZL were analyzed.

Results: The median age was 49 years (range, 13–89 years). The study involved 129 males (52.2%) and 118 females (47.8%) The most common involving site was orbit and ocular adnexa (48.6%) followed by lymph node and lymphatic organs (17.8%), bowel (9.3%), lung (6.1%), thyroid gland (4.9%), salivary gland (4.5%) in the decreasing order of frequency. Ann Abor stage I/II disease was present in 78%(167 out of 215). BM involvement was less than 10%(19 out of 211). B symptom was observed in only 2%. One and eighty-six patients out of 208 were in low or low-intermediate risk group (89%) according to international prognostic index(IPI). Eighty percents (172/215) were in low risk group in follicular lymphoma international prognostic index (FLIPI). Patients were treated with a variety of therapeutic strategies. Complete and partial remissions were achieved in 139(92%) and 8(5.3%) of the 151 stage I/II patients, respectively, with an overall response rate of 97.3%. Especially, radiation containing treatment achieved 96% CR rate (108 out of 113). In 38 patients with stage III/IV, CR and PR were achieved in 17(44.7%) and 11(28.9%). The estimated 5-year overall survival (OS) and progression free survival (PFS) were 93.8% and 70.1%, respectively. Although anthracyclin contaning regimen could achieve higher CR rate, it did not improve PFS. Stage III/IV, low hemoglobin, high/high-intermediate IPI, poor risk FLIPI and nodal MZL were poor prognostic factors for PFS in multivariate analysis.

Conclusion: NG-MZL is an indolent disease. For localized disease radiation achieved excellent local control. Anthracyclin containing chemotherapy cannot improve outcome. Both IPI and FLIPI can be applied to predict the prognosis.