CD30 expression in neoplastic T cells of follicular T cell lymphoma is a helpful diagnostic tool in the differential diagnosis of Hodgkin lymphoma

Mod Pathol. 2019 Jan;32(1):37-47. doi: 10.1038/s41379-018-0108-5. Epub 2018 Aug 23.

Abstract

Follicular T cell lymphoma is derived from follicular T-helper cells. In many cases, neoplastic T cells form rosettes around Hodgkin-Reed-Sternberg-like cells, which can lead to the misdiagnosis of classical Hodgkin lymphoma. The aim of the present study was to obtain a better understanding of this rosetting phenomenon and to recognize features that are helpful in the differential diagnosis of classical Hodgkin lymphoma. Sixteen mostly elderly follicular T cell lymphoma patients (mean 66 years) were analyzed. Fifteen of the 16 follicular T cell lymphoma cases presented with Hodgkin-Reed-Sternberg-like cells, which were CD20-positive in 27% of the cases and Epstein-Barr virus-infected in nearly all cases. Frequently, the immunophenotype of rosetting neoplastic T cells differed from the bulk neoplastic cells with less numerous T-follicular helper cell markers expressed, suggesting a modulation of T-follicular helper cell marker expression in the neoplastic T cells. In 75% of the cases, variable CD30 expression was encountered in the neoplastic T cells, likely reflecting an activation state in these cells. Hodgkin-Reed-Sternberg-like cells were positive for CCL17, and follicular T cell lymphoma tumor cells expressed its receptor CCR4 at variable intensity, thus potentially explaining the phenomenon of the tumor cells' rosetting around Hodgkin-Reed-Sternberg-like cells. In summary, this study confirms the presence of Hodgkin-Reed-Sternberg-like cells in a high number of cases of follicular T cell lymphoma, suggesting that Hodgkin-Reed-Sternberg-like cells may contribute to the development of this lymphoma. Hodgkin-Reed-Sternberg-like cells in follicular T cell lymphoma cannot reliably be differentiated from the Hodgkin-Reed-Sternberg cells of classical Hodgkin lymphoma based on their immunophenotype. In contrast, demonstration of a T-follicular helper cell phenotype with CD10 and frequent CD30 expression in the neoplastic T cell population can help to establish the diagnosis of follicular T cell lymphoma, and may even indicate CD30 as a therapeutic target for these patients.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Biomarkers, Tumor / analysis*
  • Diagnosis, Differential
  • Female
  • Hodgkin Disease / diagnosis*
  • Humans
  • Ki-1 Antigen / analysis
  • Ki-1 Antigen / biosynthesis*
  • Lymphoma, Follicular / diagnosis*
  • Male
  • Middle Aged
  • T-Lymphocytes / pathology

Substances

  • Biomarkers, Tumor
  • Ki-1 Antigen