In recognition of its unique biology and clinical behavior, DHL has been included in the 2016 revision of the World Health Organization (WHO) classification of lymphoid neoplasms as a new category of “high-grade B-cell lymphoma (HGBL) with MYC and BCL2 or BCL6 rearrangements” [2, 3]. This evidence concerns the gene MYC and high grade B-cell lymphoma.