Enteropathy-type intestinal T-cell lymphoma: clinical features and treatment of 31 patients in a single center.
OBJECTIVE
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).


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.


CONCLUSIONS
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.
Gale
J
J
Cancer Research Campaign Wessex Medical Oncology Unit, Royal South Hants Hospital, Southampton, United Kingdom. JGale@soton.ac.uk
Simmonds
P D
PD
Mead
G M
GM
Sweetenham
J W
JW
Wright
D H
DH
D000206:Actuarial Analysis; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use; D002446:Celiac Disease / Q000473:pathology; D018450:Disease Progression; D018572:Disease-Free Survival; D004750:Enteral Nutrition; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007414:Intestinal Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery / Q000628:therapy; D007421:Intestine, Small / Q000473:pathology; D016399:Lymphoma, T-Cell / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000473:pathology; D009367:Neoplasm Staging; D009752:Nutritional Status; D010288:Parenteral Nutrition; D010808:Physical Examination; D011183:Postoperative Complications; D011379:Prognosis; D012074:Remission Induction; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome