Enteropathy-type intestinal T-cell lymphoma: Clinical features and treatment of 31 patients in a single center

Purpose: We report the clinical features and treatment of 31 patients with a diagnosis of enteropathy-type intestinal T-cell lymphoma treated at the W essex Regional Medical Oncology Unit in Southampton between 1979 and 1996 ( 23 men, eight women). Patients and Methods:: Patients were identified from our lymphoma database. Details of history, physical examination, staging investigations, treatmen t, and outcome were taken from patient records. Results: Twelve patients (35%) had a documented clinical history of adult-o nset celiac disease, and a further three had histologic features consistent with celiac disease in resected areas of the small bowel not infiltrated w ith lymphoma, After diagnosis, 24 (77%) of the 31 patients were treated wit h chemotherapy; the remaining seven had surgical treatment alone. More than half were unable to complete their planned chemotherapy courses, often bec ause of poor nutritional status; 12 patients required enteral or parenteral feeding. A response to initial chemotherapy was observed in 14 patients (c omplete response, n = 10; partial response, 4). Observed complications of t reatment were gastrointestinal bleeding, small-bowel perforation, and the d evelopment of enterocolic fistulae, Relapses occurred 1 to 60 months from d iagnosis in 79% of those who responded to initial therapy. Of the total 31 patients, 26 (84%) have died, all from progressive disease or from complica tions of the disease and/or its treatment. The actuarial 1- and 5-year surv ival rates are 38.7% and 19.7%, respectively, with 1- and 5-year failure-fr ee survival rates of 19.4% and 3.2%, respectively. Conclusion: The prognosis for these patients is poor, This, in part, reflec ts late diagnosis and poor performance status at the time of presentation. The role of salvage treatments and high-dose chemotherapy at relapse is not clear, However, it is encouraging that there are five long-term survivors in our patient population. (C) 2000 by American Society of Clinical Oncolog y.