Follicular T-cell lymphoma mimicking lymphocyte-rich classic Hodgkin lymphoma: a case report of a diagnostic pitfall

• Published in: Journal of Clinical and Experimental Hematopathology Vol. 61; no. 2; pp. 97 - 101
• Main Authors: Sakakibara, Ayako, Suzuki, Yuka, Kato, Harumi, Yamamoto, Kazuhito, Sakata-Yanagimoto, Mamiko, Ishikawa, Yuichi, Furukawa, Katsuya, Shimada, Kazuyuki, Kohno, Kei, Nakamura, Shigeo, Satou, Akira, Kato, Seiichi
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society for Lymphoreticular Tissue Research 01.01.2021; JSLRT
• Subjects: Case Report; diagnostic pitfall; Follicular T-cell lymphoma; lymphocyte-rich classic Hodgkin lymphoma; programmed cell death protein 1 (PD1); RHOA mutation; diagnostic pitfall; Follicular T-cell lymphoma; programmed cell death protein 1 (PD1); lymphocyte-rich classic Hodgkin lymphoma; RHOA mutation
• ISSN: 1346-4280; 1880-9952
• DOI: 10.3960/jslrt.20052

Follicular T-cell lymphoma (FTCL), one of the nodal T-cell lymphomas with T follicular helper (TFH) phenotype, is an uncommon disease. The diagnosis of FTCL is challenging on the distinction from the morphological mimics mostly exemplified by follicular lymphoma. Here, we described a case of FTCL that mimicked lymphocyte-rich classic Hodgkin lymphoma (LRCHL). A 47-year-old male presented with cervical lymphadenopathy. The biopsy specimen demonstrated nodular lymphoid proliferation, which included scattered CD30+ CD15- CD20- PAX5 weakly+ Hodgkin and Reed-Sternberg (HRS)-like cells and a rich distribution of CD3+ CD4+ PD1+ T-cells. Epstein Barr virus was not detected in HRS-like cells, but it was detected in a small proportion of the scattered lymphocytes. The large cells were also negative for programmed cell death ligand 1, which appeared to be coincidental as described in our previous report of LRCHL. However, flow cytometry showed a CD3- CD4+ T-cell population that constituted 37.4% of all gated lymphocytes. A PCR analysis showed a clonal T-cell receptor-gamma gene rearrangement, but not a clonal immunoglobulin heavy chain gene rearrangement, and showed RHOA G17V mutation. The constellation of these findings led us to revise the diagnosis to FTCL. This result indicated that our case belonged to a relatively indolent subgroup of nodal peripheral T-cell lymphoma of TFH phenotype, which affects patients ≤60 years old, recently proposed by our group. This case report expands our understanding of the morphologic spectrum of FTCL and its clinicopathologic significance.