Enterotherapy associated T-cell lymphoma: a case report and literature review

T-cell lymphoma of the intestine is a form of extranodal, non-Hodgkins lymphoma. Enteropathy associated T-cell lymphoma (EATCL) is a unique form of T-cell lymphoma involving the gastrointestinal tract. This disorder is rare and there is no consensus on guidelines for diagnosis or treatment.

This 47 year old patient presented to the hospital with increasing abdominal girth and swelling of the lower extremities over the four days prior to admission. He complained of dyspnea on exertion, anorexia, nausea and vomiting, 40 pound weight loss over half a year, accompanied by intermittent diarrhea. On physical examination, the patient was found to have anasarca, an abdominal fluid wave with shifting dullness, mild abdominal tenderness, marked pitting edema of the extremities, and necrotic toes. A small bowel follow through revealed an abnormality in the mid-jejunum and a colonoscopy revealed two erythematous lesions near the cecum. Subsequent esophagoduodenoscopy and enteroscopy identified multiple white patchy lesions in the proximal jejunum. Biopsies were consistent with a T-cell lymphoma. The patient underwent a partial small bowel resection. Biopsies of the jejunum had ulceration and necrosis with a lymphomatous infiltrate. The lymphocytes were found to be a monoclonal CD2 positive T-cell population. A bone marrow biopsy was unremarkable. Enteropathy associated T-cell lymphoma is a rare, and this may be the first reported case of EATCL presenting as anasarca.

The etiology and pathophysiology of intestinal lymphomas, particularly enteropathy associated T-cell lymphoma, is reviewed.

EATCL is a rare and unique form of lymphoma without an exact treatment regimen which carries a high mortality rate.