NFKBIZ was originally shown to contribute to ABC-DLBCL pathogenesis by upregulating specific NF-κB target genes [108]. NFKBIZ mutations tend to cluster with BN2 -DLBCL, which also displays BCL10, PRKCB, and TRAF6 mutations, in addition to TNFAIP3 and TNIP1, potentially promoting the formation or function of the CBM protein complex. The gene discussed is BNC2; the disease is diffuse large B-cell lymphoma.