title: Peripheral T-cell lymphomas with a follicular growth pattern are derived from follicular helper T cells (TFH) and may show overlapping features with angioimmunoblastic T-cell lymphomas.
abstract: Rare cases of peripheral T-cell lymphomas with follicular growth pattern (PTCL-F) have been recently reported, and their association with t(5;9)(q33;q22) involving ITK and SYK has been suggested. However, the clinicopathologic aspects of PTCL-F are poorly described and the normal cell counterpart of this subgroup of lymphoma is still unknown. Therefore, we analyzed the pathologic, phenotypic, and cytogenetic features of a series of 30 patients (range: 33 to 88 y) that showed histopathologic features of PTCL-F in at least 1 biopsy (n=30), either at initial presentation (n=26) or at relapse (n=4). Neoplastic cells were medium-sized clear cells that were CD4+ (24/27, 89%), CD10+ (21/29, 72%), BCL-6+ (14/19, 74%), and expressed programed death-1 (27/27, 100%), CXCL13 (23/27, 85%), and ICOS (11/11, 100%), markers of follicular helper T cells (TFH). Four of 22 patients (18%) had t(5;9)(q33;q22) detected by fluorescence in situ hybridization. Patients with clinical data available had multiple lymphadenopathies (25/28, 89%), stage III to IV diseases (17/26, 65%), B symptoms (7/27, 26%), and skin lesions (6/23, 26%). Three patients with sequential biopsies disclosed clinical and histopathologic features of angioimmunoblastic T-cell lymphoma at initial presentation. Our results show that this rare form of PTCL-F (1) has an immunophenotype indicative of derivation from TFH cells, (2) is associated with t(5;9) in a proportion of cases, and (3) shows some overlapping features with angioimmunoblastic T-cell lymphoma, raising the question of a possible relationship.
authors.affiliation: INSERM, Unité 955, Equipe 9, France.
authors.forename: Yenlin
authors.initials: Y
authors.lastname: Huang
authors.forename: Anne
authors.initials: A
authors.lastname: Moreau
authors.forename: Jehan
authors.initials: J
authors.lastname: Dupuis
authors.forename: Berthold
authors.initials: B
authors.lastname: Streubel
authors.forename: Barbara
authors.initials: B
authors.lastname: Petit
authors.forename: Steven
authors.initials: S
authors.lastname: Le Gouill
authors.forename: Nadine
authors.initials: N
authors.lastname: Martin-Garcia
authors.forename: Christiane
authors.initials: C
authors.lastname: Copie-Bergman
authors.forename: Fanny
authors.initials: F
authors.lastname: Gaillard
authors.forename: Marwan
authors.initials: M
authors.lastname: Qubaja
authors.forename: Bettina
authors.initials: B
authors.lastname: Fabiani
authors.forename: Giovanna
authors.initials: G
authors.lastname: Roncador
authors.forename: Corinne
authors.initials: C
authors.lastname: Haioun
authors.forename: Marie-Hélène
authors.initials: MH
authors.lastname: Delfau-Larue
authors.forename: Teresa
authors.initials: T
authors.lastname: Marafioti
authors.forename: Andreas
authors.initials: A
authors.lastname: Chott
authors.forename: Philippe
authors.initials: P
authors.lastname: Gaulard
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015703:Antigens, CD / Q000032:analysis; D000945:Antigens, Differentiation, T-Lymphocyte / Q000032:analysis; D051017:Apoptosis Regulatory Proteins / Q000032:analysis; D001706:Biopsy; D015704:CD4 Antigens / Q000032:analysis; D054382:Chemokine CXCL13 / Q000032:analysis; D002895:Chromosomes, Human, Pair 5; D002899:Chromosomes, Human, Pair 9; D004268:DNA-Binding Proteins / Q000032:analysis; D005060:Europe; D015972:Gene Expression Regulation, Neoplastic; D015329:Gene Rearrangement, T-Lymphocyte; D005838:Genotype; D006801:Humans; D007119:Immunoblastic Lymphadenopathy / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D016130:Immunophenotyping; D017404:In Situ Hybridization, Fluorescence; D060889:Inducible T-Cell Co-Stimulator Protein; D008224:Lymphoma, Follicular / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D016411:Lymphoma, T-Cell, Peripheral / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D008875:Middle Aged; D009367:Neoplasm Staging; D015260:Neprilysin / Q000032:analysis; D010641:Phenotype; D061026:Programmed Cell Death 1 Receptor; D051560:Proto-Oncogene Proteins c-bcl-6; D006377:T-Lymphocytes, Helper-Inducer / Q000276:immunology / Q000473:pathology*; D014178:Translocation, Genetic
chemical_list: D015703:Antigens, CD; D000945:Antigens, Differentiation, T-Lymphocyte; D051017:Apoptosis Regulatory Proteins; C084103:BCL6 protein, human; D015704:CD4 Antigens; C110663:CXCL13 protein, human; D054382:Chemokine CXCL13; D004268:DNA-Binding Proteins; C556692:ICOS protein, human; D060889:Inducible T-Cell Co-Stimulator Protein; C105992:PDCD1 protein, human; D061026:Programmed Cell Death 1 Receptor; D051560:Proto-Oncogene Proteins c-bcl-6; D015260:Neprilysin