Advertisement

Indolent T-cell lymphoproliferative disease of the gastrointestinal tract

Anamarija M. Perry, Roger A. Warnke, Qinglong Hu, Philippe Gaulard, Christiane Copie-Bergman, Serhan Alkan, Huan-You Wang, Jason X. Cheng, Chris M. Bacon, Jan Delabie, Erik Ranheim, Can Kucuk, XiaoZhou Hu, Dennis D. Weisenburger, Elaine S. Jaffe and Wing C. Chan

Key points

  • Ten cases of an indolent T-cell lymphoproliferative disease of the gastrointestinal tract are reported

  • It is important to recognize this condition, as it can be mistaken for aggressive T-cell lymphoma, which may lead to unnecessary therapy

Abstract

Primary gastrointestinal (GI) T-cell lymphoma (TCL) is an infrequent and aggressive disease. However, rare indolent clonal T-cell proliferations in the GI tract have been described. We report ten cases of GI involvement by an indolent T-cell lymphoproliferative disease (T-LPD), including six men and four women with a median age of 48 years (range, 15-77 yrs.). 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 non-destructive, and composed of small mature-appearing lymphoid cells. Eight cases were CD4-/CD8+, one was CD4+/CD8-, and another was CD4-/CD8-. T-cell receptor-gamma chain gene rearrangement identified a clonal population in all ten cases. There was no evidence of STAT3 SH2 domain mutation or activation. Six patients received chemotherapy due to initial diagnosis of peripheral T-cell lymphoma, with little or no response, whereas the other four were followed without therapy. After a median follow up of 38 months (range, 9-175 months), nine patients were alive with persistent disease and one was free of disease. We propose the name "indolent T-LPD of the GI tract" for these lesions which can easily be mistaken for intestinal PTCL, and lead to aggressive therapy.

  • Submitted July 5, 2013.
  • Accepted August 26, 2013.