Nodal marginal zone B-cell lymphoma: analysis of 36 cases. Clinical presentation and treatment outcomes of nodal marginal zone B-cell lymphoma

Abstract

Nodal marginal zone B-cell lymphoma (NMZL) is a relatively uncommon type of lymphoma. Because of the rarity, the natural history and the optimal treatment modality have not been well defined. Therefore, we performed a retrospective analysis of the clinical features and treatment outcomes of NMZL. Thirty-six patients who were histologically diagnosed as NMZL were included in the analysis. Fifty-three percent of the patients had localized disease (stages I and II), and 21.2% (7/33) had bone marrow involvement at presentation. B symptom was present in only three patients (8.3%). Most patients were categorized as low or low-intermediate risk group by international prognostic index (IPI) (77.1%). Majority (94.4%) of the patients with localized disease achieved complete remission (CR) after the initial treatment. Of the seven patients with disseminated disease, who were treated with anthracycline-based chemotherapy, four patients achieved CR. Of the seven patients who received nonanthracycline-based chemotherapy, no patient achieved CR. After the median follow-up duration of 36 months, the median progression-free survival (PFS) was 3.9 (95% CI; 2.9-5.6) years, and the estimated 5-year PFS and overall survival rates were 47.2 and 82.7%, respectively. The significant predictive factors for PFS were performance status, advanced stage, and follicular lymphoma IPI (FLIPI) in this study. This clinical feature is similar to FL rather than to MZL-MALT type.

Recognizing nodal marginal zone lymphoma: recent advances and pitfalls. A systematic review

“…21 In a larger study by Arcaini et al, 9 of 38 (24%) patients with NMZL had positive serology for HCV, in one patient combined with positive hepatitis B virus (HBV) serology. 16 Some other studies confirmed an association with HCV in a smaller proportion of patients (Camacho et al and Oh et al in 20% and 5%, respectively), 13,14 but other studies did not. 15 The study by Camacho et al also showed HBV infection in 30% of patients.…”

“…[9][10][11][12][13][14][15][16][17][18][19][20] Both sexes are affected with approximately equal frequency.…”

“…19 Age, the presence of B symptoms, Ann Arbor stage, anemia, performance score, sex, race, and chemotherapy were associated with progression-free survival or event-free survival in multivariate analyses. 11,14,16 It is clear that a better understanding of this lymphoma type is needed in order to select these patients for newer, targeted therapies and to improve outcome. …”

Marginal zone lymphomas

“…[16][17][18] However, reports in the literature of nodal MZL are fewer [18][19][20][21] and most are limited to small numbers of patients. These studies also do not compare patients with nodal MZL directly to patients with extranodal or splenic MZL.…”

“…These studies also do not compare patients with nodal MZL directly to patients with extranodal or splenic MZL. 20,22 In this study, we retrospectively reviewed 275 patients diagnosed with 1 of the 3 types of MZL, to compare the prognosis of the 3 patient groups. Most of these patients (>75%) had extranodal MZL; this relative frequency is comparable to that reported in the literature.…”