Cytogenetic and pathologic characterization of MYC-rearranged B-cell lymphomas in pediatric and young adult patients

AbstractMYC-rearranged B-cell lymphoma (BCL) in the pediatric/young adult (YA) age group differs substantially in disease composition from adult cohorts. However, data regarding the partner genes, concurrent rearrangements, and ultimate diagnoses in these patients is scarce compared to that in adult cohorts. We aimed to characterize the spectrum ofMYC-rearranged (MYC-R) mature, aggressive BCL in the pediatric/YA population. A retrospective study of morphologic, immunophenotypic, and fluorescence in situ hybridization (FISH) results of patients age  ≤ 30 years with suspected Burkitt lymphoma (BL), diffuse large B-cell lymphoma (DLBCL) or high-grade B-cell lymphoma (HGBCL), and aMYC-R by FISH between 2013 –2022 was performed. Two-hundred fifty-eight cases (129 (50%) pediatric (<  18 years) and 129 (50%) YA (18–30 years)) were included. MostMYC-R BCL in pediatric (89%) and YA (66%) cases were BL. While double-hit (DH) cytogenetics (MYC withBCL2 and/orBCL6-R, HGBCL-DH) was rare in the pediatric population (2/129, 2%), HGBCL-DH increased with age and was identified in 17/129 (13%) of YA cases. Most HGBCL-DH hadMYC andBCL6-R, whileBCL2-R were rare in both groups (3/258, 1%).MYC-R without an IG partner was more common in the YA group (14/116 (12%) vs 2/128 (2%),p = 0.001). The pediatric to YA transition is characterized by decreasing frequency in BL and increasing genetic heterogeneity ofMYC-R BCL, with emergence of DH-BCL withMYC andBCL6-R. FISH to evaluate fo...
Source: Journal of Hematopathology - Category: Pathology Source Type: research