In our paper, we discuss the entities included in the large family of LBCL, focusing on the following diseases: LBLs transformed from indolent B-cell lymphomas, diffuse large B-cell lymphoma, not otherwise specified (DLBCL, NOS), double-hit/triple-hit lymphomas (DH/TH), high-grade large B-cell lymphoma, not otherwise specified (HGBCL, NOS), LBCL with IRF4 rearrangement, Burkitt lymphoma (BL) and HGBCL/LBCL with 11q aberration, pointing out, in particular, the differences between ICC and WHO-HAEM5. Here, IRF4 is linked to dermatitis herpetiformis, familial.