title: Intestinal γδ T-cell lymphomas are most frequently of type II enteropathy-associated T-cell type.
abstract: Enteropathy-associated T-cell lymphoma includes type I cases and distinctive type II cases that, according to 2008 and 2010 World Health Organization descriptions, are T-cell receptor β+. Although T-cell receptor γδ enteropathy-associated T-cell lymphomas are reported, it is unknown if they have distinctive features and if they should be categorized as enteropathy-associated T-cell lymphoma or as a mucocutaneous γδ T-cell lymphoma. To address these questions, the clinicopathologic, immunophenotypic, molecular, and cytogenetic features of 5 γδ-enteropathy-associated T-cell lymphomas were investigated. Only 1 patient had celiac disease and had type I enteropathy-associated T-cell lymphoma, and the others fulfilled the histopathologic criteria for type II enteropathy-associated T-cell lymphoma. All lacked cutaneous involvement. A celiac disease-associated HLA type was found in the patient with CD and one of four others. All were T-cell receptor γ+, T-cell receptor δ+, βF1-, CD3+, CD7+, CD5-, CD4-, and TIA-1+ with variable staining for CD2 (3/5), CD8 (2/5), Granzyme B (1/5), and CD56 (4/5). Fluorescence in situ hybridization demonstrated 9q34 gains in 4 cases, with 9q33-34 gains by single nucleotide polymorphism in 3 of these. Single nucleotide polymorphism analysis also demonstrated gains in 5q34-q35.1/5q35.1 (4/5), 8q24 (3/5), and in 32 other regions in 3 of 5 cases. Vδ1 rearrangements were identified in 4 of 4 cases with documented clonality showing the same clone in normal-appearing distant mucosa (3/3 tested cases). Thus, γδ-enteropathy-associated T-cell lymphomas share many features with other enteropathy-associated T-cell lymphoma and are mostly of type II. Their usual nonactivated cytotoxic phenotype and Vδ1 usage are features unlike many other mucocutaneous γδ T-cell lymphomas but shared with hepatosplenic T-cell lymphoma. These findings support the conclusion that a γδ T-cell origin at extracutaneous sites does not define a specific entity.
authors: University of Pittsburgh, Pittsburgh, PA 15213, USA.
authors: Amanda L
authors: AL
authors: Wilson
authors: Steven H
authors: SH
authors: Swerdlow
authors: Grzegorz K
authors: GK
authors: Przybylski
authors: Urvashi
authors: U
authors: Surti
authors: John K
authors: JK
authors: Choi
authors: Elias
authors: E
authors: Campo
authors: Massimo M
authors: MM
authors: Trucco
authors: S Branden
authors: SB
authors: Van Oss
authors: Raymond E
authors: RE
authors: Felgar
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000032:analysis; D058527:Enteropathy-Associated T-Cell Lymphoma / Q000276:immunology / Q000473:pathology*; D005260:Female; D006680:HLA Antigens / Q000276:immunology; D006801:Humans; D016130:Immunophenotyping; D017404:In Situ Hybridization, Fluorescence; D007414:Intestinal Neoplasms / Q000276:immunology / Q000473:pathology*; D016399:Lymphoma, T-Cell / Q000276:immunology / Q000473:pathology*; D008297:Male; D020411:Oligonucleotide Array Sequence Analysis; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000276:immunology*
chemical_list: D014408:Biomarkers, Tumor; D006680:HLA Antigens; D016692:Receptors, Antigen, T-Cell, gamma-delta