Spontaneously relapsing clonal, mucosal cytotoxic T-cell lymphoproliferative disorder - Case report and review of the literature

Primary T-cell lymphoma of the gastrointestinal tract is a rare and usually aggressive disorder that may be associated with celiac disease. The author s describe a unique case of a clonal proliferation of CD8+ T cells involvin g the oral mucosa, ileum, and colon of a 35-year-old man that has regressed spontaneously and recurred numerous times over a 9-year period without tre atment. The patient's symptoms were limited to occasional rectal bleeding a nd recurring painful oral ulcers. Within the intestine, these collections o f small T cells induced minimal architectural distortions and did not show extensive epitheliotrophism. Polymerase chain reaction and sequencing analy ses revealed that the identical T-cell clone has been present for more than 9 years and in different mucosal locations in this patient. This may repre sent a unique T-cell lymphoproliferative process akin to a mucosal counterp art of lymphomatoid papulosis of the skin.