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Open Access 02-04-2024 | Lymphoma | Original Article

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

Authors: Marie-France Gagnon, Frido K. Bruehl, Daniel R. Sill, Reid G. Meyer, Patricia T. Greipp, Nicole L. Hoppman, Xinjie Xu, Linda B. Baughn, Jess F. Peterson, Ellen D. McPhail, Rhett P. Ketterling, Rebecca L. King

Published in: Journal of Hematopathology

Abstract

MYC-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 of MYC-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 a MYC-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. Most MYC-R BCL in pediatric (89%) and YA (66%) cases were BL. While double-hit (DH) cytogenetics (MYC with BCL2 and/or BCL6-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 had MYC and BCL6-R, while BCL2-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 of MYC-R BCL, with emergence of DH-BCL with MYC and BCL6-R. FISH to evaluate for BCL2 and BCL6 rearrangements is likely not warranted in the pediatric population but should continue to be applied in YA BCL.

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Title: Cytogenetic and pathologic characterization of MYC-rearranged B-cell lymphomas in pediatric and young adult patients
Authors: Marie-France Gagnon
Frido K. Bruehl
Daniel R. Sill
Reid G. Meyer
Patricia T. Greipp
Nicole L. Hoppman
Xinjie Xu
Linda B. Baughn
Jess F. Peterson
Ellen D. McPhail
Rhett P. Ketterling
Rebecca L. King
Publication date: 02-04-2024
Publisher: Springer Berlin Heidelberg
Keywords: Lymphoma
Diffuse Large B-Cell Lymphoma
Diffuse Large B-Cell Lymphoma
Fluorescence in Situ Hybridization
Published in: Journal of Hematopathology
Print ISSN: 1868-9256
Electronic ISSN: 1865-5785
DOI: https://doi.org/10.1007/s12308-024-00579-6

At a glance: The STEP trials

Springer Medicine

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

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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