Pathologic and Molecular Insights in Nodal T-follicular Helper Cell Lymphomas

Authors

Mario L Marques-Piubelli
Catalina Amador
Francisco Vega

Publication Date

1-1-2023

Journal

Frontiers in Oncology

Abstract

T-follicular helper (TFH) cells are one of the T-cell subsets with a critical role in the regulation of germinal center (GC) reactions. TFH cells contribute to the positive selection of GC B-cells and promote plasma cell differentiation and antibody production. TFH cells express a unique phenotype characterized by PD-1hi, ICOShi, CD40Lhi, CD95hi, CTLAhi, CCR7lo, and CXCR5hi . Three main subtypes of nodal TFH lymphomas have been described: 1) angioimmunoblastic-type, 2) follicular-type, and 3) not otherwise specified (NOS). The diagnosis of these neoplasms can be challenging, and it is rendered based on a combination of clinical, laboratory, histopathologic, immunophenotypic, and molecular findings. The markers most frequently used to identify a TFH immunophenotype in paraffin-embedded tissue sections include PD-1, CXCL13, CXCR5, ICOS, BCL6, and CD10. These neoplasms feature a characteristic and similar, but not identical, mutational landscape with mutations in epigenetic modifiers (TET2, DNMT3A, IDH2), RHOA, and T-cell receptor signaling genes. Here, we briefly review the biology of TFH cells and present a summary of the current pathologic, molecular, and genetic features of nodal lymphomas. We want to highlight the importance of performing a consistent panel of TFH immunostains and mutational studies in TCLs to identify TFH lymphomas.

Keywords

angioimmunoblastic T cell lymphoma, follicular T helper, molecular and genetic profiling, next-generation sequencing, peripheral T cell lymphomas

DOI

10.3389/fonc.2023.1105651

PMID

36793612

PMCID

PMC9923156

PubMedCentral® Posted Date

1-30-2023

PubMedCentral® Full Text Version

Post-print

Published Open-Access

yes