Follicular peripheral T-cell lymphoma expands the spectrum of classical Hodgkin lymphoma mimics.
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.
Département de Pathologie, APHP, Groupe Henri Mondor, Créteil, France.
Julien
J
Moroch
Christiane
C
Copie-Bergman
Laurence
L
de Leval
Anne
A
Plonquet
Nadine
N
Martin-Garcia
Marie-Hélène
MH
Delfau-Larue
Valérie
V
Molinier-Frenkel
Karim
K
Belhadj
Corinne
C
Haioun
Josée
J
Audouin
Steven H
SH
Swerdlow
Teresa
T
Marafioti
Philippe
P
Gaulard
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015703:Antigens, CD / Q000378:metabolism; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001402:B-Lymphocytes / Q000378:metabolism / Q000473:pathology / Q000821:virology; D014408:Biomarkers, Tumor / Q000235:genetics / Q000378:metabolism; D002999:Clone Cells; D004273:DNA, Neoplasm / Q000032:analysis; D003937:Diagnosis, Differential; D005260:Female; D005434:Flow Cytometry; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor / Q000235:genetics; D019675:Genes, T-Cell Receptor gamma / Q000235:genetics; D004854:Herpesvirus 4, Human / Q000302:isolation & purification; D006689:Hodgkin Disease / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism; D006801:Humans; D016130:Immunophenotyping; D017404:In Situ Hybridization, Fluorescence; D008198:Lymph Nodes / Q000378:metabolism / Q000473:pathology / Q000821:virology; D008206:Lymphatic Diseases / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D008224:Lymphoma, Follicular / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics / Q000378:metabolism; D016411:Lymphoma, T-Cell, Peripheral / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D016539:Reed-Sternberg Cells / Q000378:metabolism / Q000473:pathology; D006377:T-Lymphocytes, Helper-Inducer / Q000378:metabolism / Q000473:pathology
D015703:Antigens, CD; D014408:Biomarkers, Tumor; D004273:DNA, Neoplasm