AIDS-Associated Pediatric High Grade B-cell Lymphoma with MYC and BCL2 Translocations

Abstract

Abstract"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.Citation: Msimang MZ, Ramdial PK, Kuppusamy JB, Nargan K, Sheik-Gafoor MH (2017) anterior colonic resection with colo-anal anastomosis and covering ileostomy was successfully undertaken, but the patient developed sepsis and required a relaparotomy for resection of necrotic colonic tissue. On recovery he received radiotherapy and further chemotherapy, but responded poorly and demised from multi-organ failure. Pathological featuresGross features: The lower anterior resection specimen measured 38 × 8 cm ( Figure 1A) and contained a 14 × 9 × 7 cm exophytic, creamwhite, fleshy tumor ( Figure 1B) with hemorrhagic foci, 12 cm and 3 cm from the proximal and distal excision margins, respectively. The proximal and circumferential resection margins were tumor-free. The microscopic findings confirmed an ulcerative, invasive malignancy with transmural involvement and a diffusely infiltrative pattern ( Figure 1C). The pleomorphic tumor cells were large with scant eosinophilic cytoplasm, indistinct cell borders and large, vesicular nuclei with distinctive nuclear membranes and prominent nucleoli ( Figure 1D). Brisk mitotic activity was evident. Immunohistochemically, there was bright and diffuse CD45 (Figure 2A), CD20 ( Figure 2B), BCL2 ( Figure 2C), BCL6 ( Figure 2D) and CD10 ( Figure 2E) immunopositivity. In addition, there was patchy MUM-1 ( Figure 3A) and approximately 60%, variable nuclear and cytoplasmic C-MYC immunopositivity ( Figure 3B) and a Ki-67 proliferation index of >90% ( Figure 3C). T-lymphocyte and terminal deoxynucleotidyl transferase immunomarkers were negative. Epstein Barr virus (EBV)-encoded small RNA (EBER) positivity ( Figure 3D) was confirmed on chromogen in-situ hybridization investigation.

Disseminated Double-Hit Lymphoma in a Young Adult

“…1 DHL are reported rarely in young adults, adolescents and children. 4,5 To date, there has been no reported case of DHL among the said population in the Philippines.…”