Follicular peripheral T-cell lymphoma expands the spectrum of classical Hodgkin lymphoma mimics

Am J Surg Pathol. 2012 Nov;36(11):1636-46. doi: 10.1097/PAS.0b013e318268d9ff.

Abstract

Epstein-Barr virus (EBV)-infected B cells with Reed-Sternberg-like cell (RS) features may occur in peripheral T-cell lymphomas (PTCLs), especially in angioimmunoblastic T-cell lymphoma. Here, we report 5 patients presenting with lymphadenopathy whose first biopsies demonstrated nodular lymphoid proliferations containing scattered CD30+, CD15+, EBV+ Hodgkin and Reed-Sternberg-like cells, which led to an initial diagnosis of lymphocyte-rich classical Hodgkin lymphoma. However, the uncommon clinical features and/or the occurrence of relapse as PTCL prompted review of the biopsies with expanded immunohistologic and molecular studies and revision of the diagnoses to follicular variant of PTCL (F-PTCL). All cases had atypical small to medium-sized CD3+ T cells that expressed CD10 (4/5) and the follicular helper T-cell (TFH) antigens BCL6, PD1, CXCL13, and ICOS. All demonstrated clonal T cells with a similar pattern in multiple samples from 4 patients. In 2 cases, flow cytometry demonstrated circulating lymphocytes with an abnormal sCD3+, CD4+, ICOS+ immunophenotype. Two patients had a skin rash at presentation, and 1 had B symptoms. Two of the 4 patients treated with polychemotherapy are alive at 3 and 6 years after first diagnosis. These cases highlight how some F-PTCLs may closely mimic lymphocyte-rich classical Hodgkin lymphoma requiring careful assessment of the T cells before rendering the latter diagnosis. The functional properties of TFH cells might lead to the presence of EBV-positive B blasts with RS-like features in TFH-derived PTCL such as angioimmunoblastic T-cell lymphoma and F-PTCL.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Antigens, CD / metabolism
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • B-Lymphocytes / metabolism
  • B-Lymphocytes / pathology
  • B-Lymphocytes / virology
  • Biomarkers, Tumor / genetics
  • Biomarkers, Tumor / metabolism
  • Clone Cells
  • DNA, Neoplasm / analysis
  • Diagnosis, Differential
  • Female
  • Flow Cytometry
  • Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor / genetics
  • Genes, T-Cell Receptor gamma / genetics
  • Herpesvirus 4, Human / isolation & purification
  • Hodgkin Disease / diagnosis*
  • Hodgkin Disease / genetics
  • Hodgkin Disease / metabolism
  • Humans
  • Immunophenotyping
  • In Situ Hybridization, Fluorescence
  • Lymph Nodes / metabolism
  • Lymph Nodes / pathology
  • Lymph Nodes / virology
  • Lymphatic Diseases / genetics
  • Lymphatic Diseases / metabolism
  • Lymphatic Diseases / pathology
  • Lymphoma, Follicular / diagnosis*
  • Lymphoma, Follicular / drug therapy
  • Lymphoma, Follicular / genetics
  • Lymphoma, Follicular / metabolism
  • Lymphoma, T-Cell, Peripheral / diagnosis*
  • Lymphoma, T-Cell, Peripheral / drug therapy
  • Lymphoma, T-Cell, Peripheral / genetics
  • Lymphoma, T-Cell, Peripheral / metabolism
  • Male
  • Middle Aged
  • Recurrence
  • Reed-Sternberg Cells / metabolism
  • Reed-Sternberg Cells / pathology
  • T-Lymphocytes, Helper-Inducer / metabolism
  • T-Lymphocytes, Helper-Inducer / pathology

Substances

  • Antigens, CD
  • Biomarkers, Tumor
  • DNA, Neoplasm