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

Joanna Gale, Peter D. Simmonds, Graham M. Mead, John W. Sweetenham, Dennis H. Wright

Research output: Contribution to journalArticle

264 Citations (Scopus)

Abstract

Purpose: We report the clinical features and treatment of 31 patients with a diagnosis of enteropathy-type intestinal T-cell lymphoma treated at the Wessex 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, treatment, and outcome were taken from patient records. Results: Twelve patients (35%) had a documented clinical history of adult- onset celiac disease, and a further three had histologic features consistent with celiac disease in resected areas of the small bowel not infiltrated with lymphoma. After diagnosis, 24 (77%) of the 31 patients were treated with chemotherapy; the remaining seven had surgical treatment alone. More than half were unable to complete their planned chemotherapy courses, often because of poor nutritional status; 12 patients required enteral or parenteral feeding. A response to initial chemotherapy was observed in 14 patients (complete response, n = 10; partial response, n = 4). Observed complications of treatment were gastrointestinal bleeding, small-bowel perforation, and the development of enterocolic fistulae. Relapses occurred 1 to 60 months from diagnosis in 79% of those who responded to initial therapy. Of the total 31 patients, 26 (84%) have died, all from progressive disease or from complications of the disease and/or its treatment. The actuarial 1- and 5-year survival rates are 38.7% and 19.7%, respectively, with 1- and 5-year failure-free survival rates of 19.4% and 3.2%, respectively. Conclusion: The prognosis for these patients is poor. This, in part, reflects 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 American Society of Clinical Oncology.

Original languageEnglish (US)
Pages (from-to)795-803
Number of pages9
JournalJournal of Clinical Oncology
Volume18
Issue number4
StatePublished - Feb 1 2000
Externally publishedYes

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T-Cell Lymphoma
Therapeutics
Drug Therapy
Celiac Disease
Lymphoma
Survival Rate
Recurrence
Salvage Therapy
Medical Oncology
Delayed Diagnosis
Parenteral Nutrition
Enteral Nutrition
Nutritional Status
Physical Examination
Fistula
Survivors
History
Databases
Hemorrhage

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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Enteropathy-type intestinal T-cell lymphoma : Clinical features and treatment of 31 patients in a single center. / Gale, Joanna; Simmonds, Peter D.; Mead, Graham M.; Sweetenham, John W.; Wright, Dennis H.

In: Journal of Clinical Oncology, Vol. 18, No. 4, 01.02.2000, p. 795-803.

Research output: Contribution to journalArticle

Gale, Joanna ; Simmonds, Peter D. ; Mead, Graham M. ; Sweetenham, John W. ; Wright, Dennis H. / Enteropathy-type intestinal T-cell lymphoma : Clinical features and treatment of 31 patients in a single center. In: Journal of Clinical Oncology. 2000 ; Vol. 18, No. 4. pp. 795-803.
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abstract = "Purpose: We report the clinical features and treatment of 31 patients with a diagnosis of enteropathy-type intestinal T-cell lymphoma treated at the Wessex 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, treatment, and outcome were taken from patient records. Results: Twelve patients (35{\%}) had a documented clinical history of adult- onset celiac disease, and a further three had histologic features consistent with celiac disease in resected areas of the small bowel not infiltrated with lymphoma. After diagnosis, 24 (77{\%}) of the 31 patients were treated with chemotherapy; the remaining seven had surgical treatment alone. More than half were unable to complete their planned chemotherapy courses, often because of poor nutritional status; 12 patients required enteral or parenteral feeding. A response to initial chemotherapy was observed in 14 patients (complete response, n = 10; partial response, n = 4). Observed complications of treatment were gastrointestinal bleeding, small-bowel perforation, and the development of enterocolic fistulae. Relapses occurred 1 to 60 months from diagnosis in 79{\%} of those who responded to initial therapy. Of the total 31 patients, 26 (84{\%}) have died, all from progressive disease or from complications of the disease and/or its treatment. The actuarial 1- and 5-year survival rates are 38.7{\%} and 19.7{\%}, respectively, with 1- and 5-year failure-free survival rates of 19.4{\%} and 3.2{\%}, respectively. Conclusion: The prognosis for these patients is poor. This, in part, reflects 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 American Society of Clinical Oncology.",
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