Clinicopathological features of indolent T-cell lymphoproliferative disorder in gastrointestinal tract

SHEN Long-shu; XIAO Jun; WANG Yao-hui; WANG Jie; GAO Li-li; ZHANG Yi-fen

doi:10.3969/j.issn.1007-8096.2018.06.008

Abstract：Objective To describe the clinicopathological features of indolent T-cell lymphoproliferative disorder of the gastrointestinal tract. Methods The clinical data and pathologic slides of one case were reviewed. Hematoxylin and eosin-stained slides from formalin-fixed and paraffin-embedded tissue were studied. Immunohistochemical studies were performed on sections using an automated immunostainer, as well as in situ hybridization for Epstein-Barr virus-encoded small RNAs (EBER) was performed, and PCR-based analysis for the rearrangement of T-cell receptor (TCR) genes was also performed. Results Endoscopically, the lesions presented with multiple or solitary polyps or nodules, diffuse finely nodular lesions throughout the gastric antrum and body; Histologically, the lesions were characterized by either nondestructive infiltration of the lamina propria, or focally to submucosal by small to medium-sized atypical T-cells with immunophenotyping of CD2 (+), CD3 (+), CD5 (+), CD7(+), CD4(-), CD8(+), TIA (+), GrB(-) and CD56 (-); Ki-67 proliferative index was about 5％; EBER was negative. The molecular studies for the TCR gene rearrangement confirmed a clonal lymphoproliferative disorder. The lesions featured an extremely low proliferation activity reflected by the Ki-67 proliferative index. By review of the literature, the case was diagnosed as indolent T-cell lymphoproliferative disorder of the gastrointestinal tract. The patient was in good health after symptomatic supportive treatment and followed up for 9 months. Conclusion Indolent T-cell lymphoproliferative disorder of the gastrointestinal tract might represent a novel disease featuring a peculiar indolent clinical course. It is important to be aware of this disease for the correct diagnosis and optimal management.