Indolent T-cell lymphoproliferative disease of the gastrointestinal tract BLOOD Perry, A. M., Warnke, R. A., Hu, Q., Gaulard, P., Copie-Bergman, C., Alkan, S., Wang, H., Cheng, J. X., Bacon, C. M., Delabie, J., Ranheim, E., Kucuk, C., Hu, X., Weisenburger, D. D., Jaffe, E. S., Chan, W. C. 2013; 122 (22): 3599-3606

Abstract

Primary gastrointestinal (GI) T-cell lymphoma is an infrequent and aggressive disease. However, rare indolent clonal T-cell proliferations in the GI tract have been described. We report 10 cases of GI involvement by an indolent T-cell lymphoproliferative disease, including 6 men and 4 women with a median age of 48 years (range, 15-77 years). Presenting symptoms included abdominal pain, diarrhea, vomiting, food intolerance, and dyspepsia. The lesions involved oral cavity, esophagus, stomach, small intestine, and colon. The infiltrates were dense, but nondestructive, and composed of small, mature-appearing lymphoid cells. Eight cases were CD4(-)/CD8(+), 1 was CD4(+)/CD8(-), and another was CD4(-)/CD8(-). T-cell receptor-? chain gene rearrangement identified a clonal population in all 10 cases. There was no evidence of STAT3 SH2 domain mutation or activation. Six patients received chemotherapy because of an initial diagnosis of peripheral T-cell lymphoma, with little or no response, whereas the other 4 were followed without therapy. After a median follow-up of 38 months (range, 9-175 months), 9 patients were alive with persistent disease and 1 was free of disease. We propose the name "indolent T-LPD of the GI tract" for these lesions that can easily be mistaken for intestinal peripheral T-cell lymphoma, and lead to aggressive therapy.

View details for DOI 10.1182/blood-2013-07-512830

View details for Web of Science ID 000329726200015

View details for PubMedID 24009234

View details for PubMedCentralID PMC3837508