In the present study, 47% of MZL presented pathogenic variants, involving TNFAIP3 (n = 6, 32%), KMT2D (n = 3, 16%), EP300 (n = 4, 21%), NOTCH2 (n = 3, 16%), and CARD11 (n = 2, 10.5%). This evidence concerns the gene TNFAIP3 and marginal zone lymphoma.