Marginal zone B-cell lymphomas (MZL) represent a heterogeneous group of indolent lymphomas, comprising of MALT lymphomas, nodal MZL and splenic MZL. Currently, there has been no widely accepted prognostic indices for these lymphomas. Recently, the International Extranodal Lymphoma Study Group (IELSG) developed a novel simplified MALT prognostic index (MALT-IPI)1, which was subsequently validated by a western cohort of MALT cases. To date, there has not been an assessment of the applicability of this score in other subtypes of MZL or in the Asian population, where geographic and ethnical differences may influence disease epidemiology and outcomes. Our study aimed to 1) Evaluate clinical characteristics and survival outcomes for a large cohort of Southeast Asian patients with MZL who were diagnosed between 1993 - 2018 and ii) Assess the prognostic significance of the MALT-IPI for this patient cohort.
Patients and Methods:
We retrospectively studied 286 consecutive patients with newly diagnosed MZL treated between 1993 to 2016 at 2 tertiary cancer centres in Singapore. Data on clinical parameters, treatments, response, and survival were evaluated. In addition, all patients with available data were classified according to the MALT-IPI (N =236, including MALT lymphomas N= 190 and nodal/splenic MZL, N= 46) and the prognostic significance of this score was evaluated.
Among 286 patients with MZL, 230 patients (80%) had MALT lymphomas, 35 (12%) had nodal MZL and 21 (7%) had splenic MZL. The median age was 60 years (range 15- 94), 49% of the patients were male and 183 patients (64%) had Stage I-II disease. The majority of the patients were managed by either radiotherapy (N= 77, 27%), chemo and/or immunotherapy (N= 76, 27%), watchful waiting (N-= 46, 16%) or antibiotics (N= 33, 11.5%).With a median follow-up of 46 mths (range 1 - 266 mths) for all patients, the 5 yr-PFS for the cohort was 59.8 %. Progression free survival was significantly better for the MALT subgroups compared to the nodal and splenic MZL subgroups ( 5 yr PFS of 63.6 vs 49.7 % vs 30.5 % respectively, p=0.002). The 5-yr OS was excellent at 86.7% and there were no differences between the MALT, nodal MZL and splenic MZL cohorts (5-yr PFS 87.1 vs 85.3% vs 86.4, p=0.667). Age, ECOG status and elevated LDH were prognostic for PFS on univariate analysis but none has significant impact on multivariate analysis. No other baseline clinical characteristic (including gender and disease stage) was significantly associated with PFS. Age and ECOG was significantly associated with OS on both univariate and multivariate analysis.
In our cohort, the MALT-IPI was a significant prognostic marker with ability to discriminate different prognostic groups with respect to PFS and OS. The 5-year progression-free survival rate for patients who had MALT-IPI scores of 0, 1 or 2-3 were 78%, 58% and 42%, respectively (p<0.001). The 5-year OS rates for patients who had MALT-IPI scores of 0, 1 or 2-3 were 98% , 89% and 68% respectively (p <0.001). Table 1 and Figure 1A and B summarises the survival endpoints (PFS and OS) according to the MALT-IPI in our patient cohort.
We demonstrated the prognostic applicability of the MALT-IPI to identify patients at risks of progression or death, in a large cohort of Asian patients with MZL. Patients with high risk MALT-IPI appear to be associated with poor outcomes and may be candidates for novel treatment approaches.
Jeyasekharan:AstraZeneca: Other: Collaboration; Merck Sharp & Dohme: Honoraria; PerkinElmer: Other: Collaboration; Janssen: Research Funding. Goh:Johnson & Johnson: Consultancy, Honoraria; Novartis: Consultancy, Honoraria; Astellas: Consultancy, Honoraria; Gilead: Consultancy, Honoraria; Sanofi-Aventis: Consultancy, Honoraria; Bristol-Myers-Squibb: Consultancy, Honoraria; Amgen: Consultancy, Honoraria; Pfizer: Consultancy, Honoraria.
Asterisk with author names denotes non-ASH members.