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Intestinal T cell lymphomas are rare, but highly aggressive in their clinical course. Generally diagnosed in advanced stages and presenting as surgical emergencies, they also respond poorly to standard anti-lymphoma therapies. Since these lymphomas are still not well characterized, we aimed our retrospective research at the evaluation of clinical features and treatment response in patients with intestinal T cell lymphomas diagnosed between February 1996 and November 2004. Fifteen patients were identified from the Department of Pathology database at the Institute of Oncology Ljubljana. Details of history, physical examination, staging investigation, treatment and outcome were taken from patient records. Ten (67%) patients had enteropathy-associated T cell lymphoma (EATL) and 5 (33%) had peripheral T cell lymphoma (PTCL). Surgery was performed on 11 patients with 8 cases on an emergency basis; all visible disease was resected in only 3 patients. In the continuation of treatment, all 15 patients received chemotherapy (predominantly CHOP). Six patients were treated with field radiotherapy as a part of the first treatment. In total, complete response was achieved in 6 patients (40%) with a median duration of 5.3 months (range, 2 to 12 months), stable disease in 3 patients (20%), and progressive disease in 6 patients (40%). Results were better in patients with limited disease. The most frequent treatment complications were small bowel perforation, obstruction, gastrointestinal bleeding, and infection. Even though some patients underwent second- and third-line treatment, 13 (87%) of the total 15 patients died from progressive disease or complications of treatment. The actuarial 1- and 5-year survival rates were 33% and 9%, respectively. The prognosis and standard treatment of patients with intestinal T cell lymphomas are unsatisfactory with only a few long-term survivors. Therefore, earlier diagnosis and the development of more effective treatments are urgently required to improve the prospects of these patients.

A single-center report on clinical features and treatment response in patients with intestinal T cell non-Hodgkin's lymphomas