High-grade B-cell lymphoma with 11q aberrations (HGBL-11q) is a new mature high-grade B-cell lymphoma (HGBL) to be described in the World Health Organization Classification 5 th edition (WHO-5). 1 HGBL-11q is a relatively rare B-cell lymphoma that exhibits a Burkitt-like or an intermediate to blastoid appearance but has 11q aberrations without MYC translocation. The concept of HGBL originated from B-cell lymphoma (BCL), unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma (BL) (BCL-U), which was proposed as a provisional entity in the WHO 4 th edition (WHO-4). 2 This category was thought to apply to aggressive lymphomas with inferior prognosis, such as BL without MYC translocation, atypical BL with marked nuclear atypia, BL with BCL2 expression, and double-hit lymphoma, which had been discussed previously. In the revised 4th edition of WHO (WHO-4R), BCL-U was reclassified as HGBL with MYC and BCL2 and/or BCL6 rearrangements, and HGBL, not otherwise specified (NOS). 3 HGBL, NOS included cases that could not be morphologically, immunohistochemically, or genetically classified as BL, Burkitt-like lymphoma with 11q aberration (BLL-11q), or other aggressive BCLs. HGBL without MYC translocation with BLL-11q was tentatively designated as a separate disease as BLL-11q in WHO-4R; BLL-11q will be changed to HGBL-11q in WHO-5.BL is an aggressive lymphoma of well-known origin in the germinal center dark zone, characterized by marked cell proliferation due to MYC translocation. The dense, cohesive growth pattern of medium-sized tumor cells and the "starry sky" appearance produced by scattered macrophages are characteristic of BL. BLL-11q was established because 11q aberrations were found in MYC-negative BL and post-transplant-associated BL. [4][5][6] Several studies of HIV-associated MYC-negative BL with 11q aberrations have been reported. 7,8 Recent studies have reported that 11q aberrations were not limited to the Burkitt-like morphology cases, but were also found in BL, HGBL, and DLBCL. 7,9,10 Regarding genetic variation, HGBL-11q was more similar to germinal center B-cell (GCB) type DLBCL than to BL, 10 and it has