Composite lymphoma composed of follicular lymphoma and nodal T-follicular helper cell lymphoma: report of 3 cases highlighting histopathologic zonation of each neoplastic component
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2026-03-03
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<jats:title>Abstract</jats:title> <jats:sec> <jats:title>Objectives</jats:title> <jats:p>Composite lymphoma (CL), composed of follicular lymphoma (FL) and nodal T-follicular helper cell lymphoma (nTFHL), is an uncommon and diagnostically challenging entity. We present a small series of such cases to characterize their clinicopathologic and diagnostic features.</jats:p> </jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>We retrospectively analyzed 3 CL cases compared with 6 control cases of FL with expanded reactive T-follicular helper cells.</jats:p> </jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>Histologically, all 3 CL cases demonstrated geographic zonation of the 2 neoplastic components, with the B-cell lymphoma residing in follicle centers (B-zones) and the T-cell neoplasm confined to perifollicular/interfollicular areas (T-zones), in contrast to a predominantly (83%) intrafollicular distribution of T-follicular helper cells in the control cases. In all CL cases, FL was suggested by histopathologic features, and the diagnosis was supported by flow cytometry. All 3 cases (100%) showed cytologic atypia and immunophenotypic aberrancy in the T-cell component, whereas none (0%) were observed in the control group. In 2 cases (66.7%), scattered Epstein-Barr virus–positive cells were seen in the T-zone, suggesting latent infection in bystander cells, again compared to none (0%) in the control. Genomic sequencing was performed in 2 cases, both (100%) showing pathogenic mutations associated with nTFHL, while none (0%) of the controls showed such mutations. Biclonality was confirmed by B-cell and T-cell receptor gene rearrangement analyses in all 3 CL cases. All patients with CL presented with an aggressive clinical course.</jats:p> </jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>This series highlights the unique histopathologic characteristics of CL and underscores the importance of a multifaceted approach to diagnosis.</jats:p> </jats:sec>
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Zhao, Yue, Ibrahim Hajjali, Yaping Ju, Luis Carrillo, Mark Feng, Qianze Dong, Dongjiang Chen, Imran Siddiqi, et al. (2026). Composite lymphoma composed of follicular lymphoma and nodal T-follicular helper cell lymphoma: report of 3 cases highlighting histopathologic zonation of each neoplastic component. Am J Clin Pathol, 165(3). 10.1093/ajcp/aqag012 Retrieved from https://hdl.handle.net/10161/34284.
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Luis Felipe Carrillo
My academic interests include flow cytometry, myeloid neoplasms, lymphoma diagnosis, cutaneous lymphomas, and Epstein Barr virus. In addition to my clinical duties, I have a strong interest in medical education, which includes teaching medical students, residents, and fellows.
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