NFKB1 and BENTA disease: B-cell expansion with nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB) and T-cell anergy syndrome (BENTA) is a rare disorder featured by constitutive activation of the NF-κB signaling pathway leading to EBV-driven polyclonal B-cell LP manifesting as lymphocytosis, splenomegaly, and lymphadenopathy (33).