A 63-year-old man presented with a perforation of the small intestine. A diagnosis of intestinal T-cell lymphoma (ITCL) was made from CD (cluster differentiation) 3 positivity and a rearrangement of T-cell receptor genes. The tumor also expressed CD56, which suggests it belongs to a rare subtype derived from activated cytotoxic intraepithelial T lymphocytes. Although the prognosis of ITCL has been considered to be very poor irrespective of CD56 positivity, complete remission was achieved in this case by high dose chemotherapy followed by autologous peripheral blood stem cell transplantation (auto-PBSCT) even after relapse. Auto-PBSCT in the earlier stage of the disease might improve the prognosis.
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