Recurrent gain-of-function mutations in the genes encoding CBM proteins or their upstream regulators result in constitutive CBM-dependent NF-κB activation and have been detected in a wide range of lymphoid malignancies including ABC DLBCL, MCL, MALT lymphoma, acute T-cell leukemia/lymphoma (ATLL), and Sézary syndrome [16,23,134,135]. The gene discussed is NFKB1; the disease is MALT lymphoma.