Msimang MZ, Ramdial PK, Kuppusamy JB, Nargan K and Mohamed H Sheik-Gafoor
“Double hit” lymphomas (DHLs) are now included as a distinct entity in the 2016 revised World Health Classification of lymphoid neoplasms under the rubric “High-grade B-cell lymphoma (HGBCL), with MYC and BCL2 and/or BCL6 translocations”. While DHL/HGBCL with MYC and BCL2 translocations accounts for approximately 10% of all lymphomas with the phenotypic and immunophenotypic features of diffuse large B-cell lymphoma (DLBCL), to date, the entity is unreported in children <12 years and in patients with AIDS. In reporting a DHL/HGBCL with MYC and BCL2 translocations in a 10 year old boy, we contextualize, for the first time, its occurrence and clinicopathological profile in the context of pediatric HIV infection and AIDS. In so doing, the defining features of DHL/ HGBCL with MYC and BCL2 translocations in the updated 2016 WHO classification are discussed. In addition, the clinicopathological features and therapeutic approaches in children and adults and related challenges thereof, are highlighted. Finally, the spectrum of AIDS-associated lymphomas in children and adults are tabulated and the associated diagnostic and therapeutic challenges are addressed briefly.
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