open access

Vol 15, No 2 (2013)
Case reports
Published online: 2014-09-23
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Jejunal perforation with obstruction due to enteropathy-associated T-cell lymphoma subtype II

Mateusz Majewski, Zbigniew Lorenc, Bartosz Pułtorak, Sławomir Kozieł
Chirurgia Polska 2013;15(2):146-150.

open access

Vol 15, No 2 (2013)
Case reports
Published online: 2014-09-23

Abstract

T-cell lymphomas are a group of rare diseases with extremely severe course. One of the forms is enteropathy-associated T-cell lymphoma (EATL), occurring with a frequency of about 0.5 to 1 per million population. Two forms of lymphoma are known: EATL 1 — pleomorphic, which is closely associated with celiac disease, and EATL 2 — monomorphic rarely or not at all associated with intestinal disorder. The most common complication is perforation and intestinal obstruction. EATL occurs in the jejunum or ileum (much less frequently is located in the stomach or duodenum) and this leads to difficulties in correct diagnosis of the disease. Present article shows one of the cases of EATL subtype II in a 36-year-old woman. Enteropathy-associated T-cell lymphoma was located in the jejunum, and was diagnosed after surgery. There is still no effective treatment despite a lot of research. It seems that the CHOP chemotherapy remains the standard of care, which however often does not provide desired effects. Surgical treatment should be confined to patients in whom haemorrhage, obstruction, perforation or extreme discomfort caused by tumour mass occured. The prognosis of patients with detected enteropathy-associated T-cell lymphoma is poor — much worse than in the case of B-cell lymphomas. Overall survival in these cases is estimated at 7 months.

Abstract

T-cell lymphomas are a group of rare diseases with extremely severe course. One of the forms is enteropathy-associated T-cell lymphoma (EATL), occurring with a frequency of about 0.5 to 1 per million population. Two forms of lymphoma are known: EATL 1 — pleomorphic, which is closely associated with celiac disease, and EATL 2 — monomorphic rarely or not at all associated with intestinal disorder. The most common complication is perforation and intestinal obstruction. EATL occurs in the jejunum or ileum (much less frequently is located in the stomach or duodenum) and this leads to difficulties in correct diagnosis of the disease. Present article shows one of the cases of EATL subtype II in a 36-year-old woman. Enteropathy-associated T-cell lymphoma was located in the jejunum, and was diagnosed after surgery. There is still no effective treatment despite a lot of research. It seems that the CHOP chemotherapy remains the standard of care, which however often does not provide desired effects. Surgical treatment should be confined to patients in whom haemorrhage, obstruction, perforation or extreme discomfort caused by tumour mass occured. The prognosis of patients with detected enteropathy-associated T-cell lymphoma is poor — much worse than in the case of B-cell lymphomas. Overall survival in these cases is estimated at 7 months.
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Keywords

enteropathy, T-cell lymphoma, EATL, celiac disease

About this article
Title

Jejunal perforation with obstruction due to enteropathy-associated T-cell lymphoma subtype II

Journal

Chirurgia Polska (Polish Surgery)

Issue

Vol 15, No 2 (2013)

Pages

146-150

Published online

2014-09-23

Bibliographic record

Chirurgia Polska 2013;15(2):146-150.

Keywords

enteropathy
T-cell lymphoma
EATL
celiac disease

Authors

Mateusz Majewski
Zbigniew Lorenc
Bartosz Pułtorak
Sławomir Kozieł

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