International Prognostic Index for marginal zone lymphomas requiring systemic treatment
The prospective FIL NF10 trial, at 65 centers in Europe and South America, included patients requiring systemic treatment for marginal zone lymphoma (MZL). Five baseline factors were identified that were associated with inferior progression-free survival: LDH above upper limit of normal, hemoglobin < 12 g/dl, absolute lymphocyte count ≤ 1 x 109/l, platelets < 100 x 109/l, and MZL subtype (nodal or disseminated). The MZL International Prognostic Index (MZL-IPI) combined these factors, defining a low-risk group (0 factors; 27% of patients), intermediate-risk group (1 or 2 factors; 57%), and a high-risk group (3 or more factors:16%). MZL-IPI was prognostic for progression-free survival both in the NF10-cohort (A) and in the US validation cohort (B). In both cohorts MZL-IPI also was prognostic for overall survival. The authors conclude that MZL-IPI is a new prognostic score for use in all patients with MZL considered for systemic treatment.