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

• DOI: 10.1097/pas.0b013e3181971591
• Publication Date: 2009-05-21T10:10:50Z
• Authors: Yenlin Huang; Anne Moreau; Jehan Dupuis; Berthold Streubel; Barbara Petit; Steven Le Gouill; Nadine Martin-Garcia; Christiane Copie-...; Fanny Gaillard; Marwan Qubaja; Bettina Fabiani; Giovanna Roncador; Corinne Haioun; Marie-Hélène Delf...; Teresa Marafioti; Andreas Chott; Philippe Gaulard
• Keywords: Antigens, Differentiation, T-Lymphocyte; Helper-Inducer; Biopsy; [SDV.BBM]Life Sciences [q-bio]/Biochemistry; MESH: Antigens, CD4; MESH: Immunoblastic Lymphadenopathy; Peripheral; MESH: Genotype; MESH: Lymphoma, Follicular; MESH: Biopsy; MESH: Aged, 80 and over; 0302 clinical medicine; follicular helper T cell.; 80 and over; MESH: In Situ Hybridization, Fluorescence; follicular helper T cell; MESH: Antigens, CD; peripheral T-cell lymphoma; In Situ Hybridization, Fluorescence; MESH: Aged; Aged, 80 and over; MESH: Middle Aged; MESH: Neprilysin; 600; MESH: Gene Expression Regulation, Neoplastic; MESH: Neoplasm Staging; MESH: Lymphoma, T-Cell, Peripheral; MESH: Gene Expression Regulation; MESH: Gene Rearrangement, T-Lymphocyte; MESH: Translocation, Genetic; CD; 3. Good health; DNA-Binding Proteins; Europe; Gene Expression Regulation, Neoplastic; Differentiation; MESH: Translocation; CD4 Antigens; Chromosomes, Human, Pair 5; MESH: Antigens; MESH: Chemokine CXCL13; Pair 5; Chromosomes, Human, Pair 9; Human; Pair 9; MESH: Chromosomes, Human, Pair 5; Adult; MESH: Immunophenotyping; Genotype; MESH: Gene Rearrangement; 610; MESH: Phenotype; Gene Rearrangement, T-Lymphocyte; Fluorescence; angioimmunoblastic T-cell lymphoma; Immunophenotyping; 03 medical and health sciences; MESH: In Situ Hybridization; Genetic; Antigens, CD; [SDV.BBM] Life Sciences [q-bio]/Biochemistry, Molecular Biology; Humans; [SDV.BBM]Life Sciences [q-bio]/Biochemistry, Molecular Biology; MESH: T-Lymphocytes, Helper-Inducer; Aged; Neoplastic; MESH: Humans; MESH: Apoptosis Regulatory Proteins; Follicular; MESH: Adult; T-Cell; Chemokine CXCL13; CD4; MESH: T-Lymphocytes; T-Lymphocyte; MESH: Antigens, Differentiation, T-Lymphocyte; Immunoblastic Lymphadenopathy; MESH: Chromosomes; MESH: Europe; MESH: Lymphoma; MESH: Chromosomes, Human, Pair 9; Apoptosis Regulatory Proteins; MESH: DNA-Binding Proteins

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.