High-grade B-cell lymphomas (HGBCL) represent a heterogeneous group of very rare mature B-cell lymphomas. The 4th revised edition of the WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues (WHO-HAEM) previously defined two categories of HGBCL: the so-called double-hit (DHL) and triple-hit (THL) lymphomas, which were related to forms harboring MYC and BCL2 and/or BCL6 rearrangements, and HGBCL, NOS (not otherwise specified), corresponding to entities with intermediate characteristics between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma (BL), without rearrangement of the MYC and BCL2, and/or BCL6 genes. In the 5th edition of the WHO-HAEM, DHL with MYC and BCL2 rearrangements or THL were reassigned as DLBCL/HGBCL with MYC and BCL2 rearrangements (DLBCL/HGBL-MYC/BCL2), whereas the category HGBCL, NOS remains unchanged.
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