KEY TAKEAWAYS
- The FIL and SPORE-MER study analyzed data from participants enlisted in the NF10 study to create a predictive score for MZL.
- The study involved adult patients who had started systemic therapy and were classified as SMZL, ENMZL, NMZL, or dissMZL.
- The study concluded that MZL-IPI better predicts patient outcomes than other markers and is reliable for overall survival.
Marginal zone lymphomas (MZL) are a type of lymphoma encompassing three subtypes: extranodal (ENMZL), nodal (NMZL), and splenic (SMZL). Although each subtype has been studied independently, MZL is typically evaluated as a whole in clinical trials. Therefore, having a prognostic score for MZL as a unified entity would be highly beneficial.
The study involved patients (pts) enrolled in the NF10 observational study, including adult pts with MZL who had started systemic therapy and were classified as SMZL, ENMZL, NMZL, or disseminated MZL (dissMZL). The primary endpoint was progression-free survival (PFS), calculated from the start of treatment. Researchers applied the same inclusion criteria and model to an independent cohort of pts from the University of Iowa/Mayo Clinic Lymphoma Specialized Program of Research Excellence (SPORE) Molecular Epidemiology Resource (MER).
From the NF10 study, 501 eligible pts were identified, comprising 166 SMZL (33%), 197 ENMZL (39%), 60 NMZL (12%), and 78 dissMZL (16%). 40% of pts were over 70 years old at the time of diagnosis, 80% were diagnosed with stage III-IV, 31% had elevated LDH levels, 41% had Hb less than 12 g/dl, 20% had lymphopenia (absolute lymphocyte count less than 1 × 109/L), and 14% had platelets less than 100 × 109/L. After 61 months of follow-up, the 5-year PFS rate was 72% (95% CI 68%–76%). The final multivariate model identified elevated LDH levels, anemia, lymphopenia, thrombocytopenia, and subtype (NMZL or dissMZL) as independent factors associated with a lower PFS rate.
A prognostic model was built based on five factors that classified pts into low (LRG, 0 factors, 27%), intermediate (IRG, 1–2 factors, 57%), and high (HRG, 3+ factors, 16%) risk groups. The low-risk group pts had an 85% 5-year progression-free survival rate (PFS). In comparison, the intermediate-risk group had a rate of 66%, and the high-risk group had a rate of 37% with c-Harrell = 0.64 and robust internal validation and calibration. Patients in the intermediate-risk group had a hazard ratio of 2.30 (95% CI 1.39-3.80), while those in the high-risk group had a hazard ratio of 5.41 (95% CI 3.12-9.38), both indicating inferior PFS compared to the low-risk group. In the MER cohort of 192 MZL pts, the 5-year PFS rate was 57% (95% CI 51-64%). On application of the MZL-IPI to the MER, pts were classified into three categories: LRG, IRG, and HRG, with 21%(41), 59%(113), and 20%(38) falling under each category, respectively.
The study demonstrated that the MZL-IPI had a significant association with PFS (log-rank test p = 0.043; c-Harrell = 0.60, 95% CI 0.55–0.66); compared to the LRG, the IRG (HR = 1.57, 95% CI 0.97–2.54) and HRG (HR = 2.04, 95% CI 1.15–3.62) had inferior PFS. The MZL-IPI outperformed IPI, FLIPI, and MALT-IPI in predicting and distinguishing patient outcomes and was prognostic for overall survival in both the training and validation studies.
MZL-IPI is a validated prognostic score that should be used for all MZL pts undergoing a systemic treatment evaluation.
Source: https://onlinelibrary.wiley.com/doi/10.1002/hon.3163_63
Clinical Trial: https://classic.clinicaltrials.gov/ct2/show/NCT02904577
Luminari, S., Bommier, C., Fabbri, N., Nizzoli, M. E., Maurer, M. J., Tarantino, V., Ferrero, S., Rattotti, S., Merli, M., Ferrari, A., Murru, R., Khurana, A., Mwangi, R., Deodato, M., Giudice, I. D., Cencini, E., Re, F., Visco, C., Feldman, A. L., Arcaini, L. MARGINAL ZONE LYMPHOMA INTERNATIONAL PROGNOSTIC INDEX (MZL-IPI): A PROGNOSTIC SCORE FOR THE ENTIRE SPECTRUM OF MARGINAL ZONE LYMPHOMAS. A FIL AND SPORE-MER STUDY. Hematological Oncology, 41, 102-103. https://doi.org/10.1002/hon.3163_63.
