The NFKBIE Y254fs mutation has been reported in aggressive chronic lymphocytic leukemia (CLL), Hodgkin lymphoma (HL), DLBCL, primary mediastinal B-cell lymphoma (PMBCL) and a few cases of low-grade B-cell NHL, including follicular lymphoma (FL), EMZL [19], and SMZL [11,20]. Here, NFKBIE is linked to B-cell chronic lymphocytic leukemia.