Intestinal ?? T-cell lymphomas are most frequently of type II enteropathy-associated T-cell type

• Published in: Human pathology Vol. 44; no. 6; p. 1131
• Main Authors: Wilson, Amanda L, Swerdlow, Steven H, Przybylski, Grzegorz K, Surti, Urvashi, Choi, John K, Campo, Elias, Trucco, Massimo M, van Oss, S Branden, Felgar, Raymond E
• Format: Journal Article
• Language: English
• Published: Philadelphia Elsevier Limited 01-06-2013
• Subjects: Automation; Cloning; Deoxyribonucleic acid; DNA; Genes; Tumors
• ISSN: 0046-8177; 1532-8392
• DOI: 10.1016/j.humpath.2012.10.002

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?+. Although T-cell receptor??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??T-cell lymphoma. To address these questions, the clinicopathologic, immunophenotypic, molecular, and cytogenetic features of 5??-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?+, T-cell receptor?+,?F1?, 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. V?1 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,??-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 V?1 usage are features unlike many other mucocutaneous??T-cell lymphomas but shared with hepatosplenic T-cell lymphoma. These findings support the conclusion that a??T-cell origin at extracutaneous sites does not define a specific entity.