Peripheral T-cell Lymphomas of Follicular T-Helper Cell Derivation With Hodgkin/Reed-Sternberg Cells of B-cell Lineage: Both EBV-positive and EBV-negative Variants Exist Journal Article

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Authors: Nicolae, A.; Pittaluga, S.; Venkataraman, G; Vijnovich-Baron, A.; Xi, L.; Raffeld, M.; Jaffe, E. S.
Article Title: Peripheral T-cell Lymphomas of Follicular T-Helper Cell Derivation With Hodgkin/Reed-Sternberg Cells of B-cell Lineage: Both EBV-positive and EBV-negative Variants Exist
Abstract: Peripheral T-cell lymphomas (PTCLs) are functionally and morphologically complex. Epstein-Barr virus (EBV)-positive B cells have been reported in angioimmunoblastic T-cell lymphoma (AITL) and other PTCLs and may mimic Hodgkin/Reed-Sternberg (HRS) cells, but EBV-negative HRS-like B cells have not been described. We wished to assess the nature of the PTCL associated with HRS-like cells and to determine whether EBV-negative HRS-like cells may be seen. We identified 57 PTCL cases reported as containing HRS-like cells. These included 32 AITL, 19 PTCL, not otherwise specified (NOS), 3 PTCL-NOS, follicular variant, 1 PTCL-NOS, T-zone variant, and 2 adult T-cell leukemia/lymphoma cases. All patients were adults with a median age of 63 and presented with lymphadenopathy. The male:female ratio was 31:26 (1.2:1). Clonal TRG rearrangement was detected in 46/53 cases. Six of 38 cases had a concomitant clonal immunoglobulin gene rearrangement. In 52/57 cases the HRS cells were positive for EBV. Five cases, 3 classified as AITL and 2 as PTCL-NOS, follicular variant, contained HRS-like cells negative for EBV. All PTCLs with EBV-negative HRS cells had a T follicular helper cell immunophenotype. The neoplastic T cells expressed CD3, CD4, and PD-1 and formed rosettes around the HRS-like cells. The HRS-like cells were positive for CD20 (variable intensity), PAX5, CD30, and CD15 (4/5). We conclude that both EBV-positive and EBV-negative HRS-like B cells may occur in the background of PTCL; caution is needed to avoid misdiagnosis as classical Hodgkin lymphoma. The close interaction between the HRS-like cells and the rosetting PD-1-positive T cells suggests a possible pathogenetic role in this phenomenon and provides new insights into the abnormal B-cell proliferations that occur in the context of TFH malignancies.
Journal Title: The American Journal of Surgical Pathology
Volume: 37
Issue: 6
ISSN: 1532-0979; 0147-5185
Publisher: Unknown  
Journal Place: United States
Date Published: 2013
Start Page: 816
End Page: 826
Language: eng
DOI/URL:

10.1097/PAS.0b013e3182785610

10.1097/PAS.0b013e3182785610
Notes: ID: 12919; JID: 7707904; NIHMS420738; OID: NLM: NIHMS420738 [Available on 06/01/14]; OID: NLM: PMC3654051 [Available on 06/01/14]; PMCR: 2014/06/01 00:00; ppublish