Abstract

Intestinal non-Hodgkin's T-cell lymphoma (NHTCL) is a rare, extranodal manifestation of lymphoma. We report a case of a 67 year old man with a history of dyslipidemia, drug-induced neutropenia and low grade B-cell lymphoproliferative disorder (most consistent with B cell chronic lymphocytic leukemia) who presented with a two week history of melena and abdominal pain. An EGD was performed and did not reveal a bleeding source. The patient continued to have dark stool, hematochezia and progressive anemia, thus a colonoscopy was performed. Multiple 5mm ulcers were noted in the cecum, ascending and transverse colon on colonoscopy. Biopsies revealed an aggressive NHTCL. Cytology showed strong expression of CD2 and CD3 with absent expression of CD5, CD7, CD34 and TdT. An aggressive proliferation index of 75-80% was noted.Figure 1Figure 2Figure 3FDG-PET scan of the abdomen/pelvis revealed multiple foci of increased metabolic activity located in the abdomen, particularly associated with small bowel loops and mesenteric lymph nodes. A bone marrow biopsy was obtained and displayed kappa restricted CD5 coexpression consistent with history of small B-cell lymphoma. Bone marrow showed no immunohistochemical evidence of NHTCL, confirming the diagnosis of primary intestinal lymphoma in accordance with Dawson criteria.1 The patient was evaluated by oncology services at two separate centers, and it was agreed that the patient had primary NHTCL. He was placed on a clinical trial of CHOP-Belinostat. Three months after diagnosis, the patient developed severe, acute abdominal pain and was found to have bowel perforation. Laparotomy revealed extensive tumors in the small bowel, of which several had perforated. His clinical course was complicated and after a prolonged ICU stay, he was transitioned to comfort measures only and died shortly thereafter. Primary gastrointestinal (GI) lymphoma is rare, comprising 1-4% of all GI malignancies, of which less than 10% are of T-cell origin.1 We suspect this patient had Enteropathy Associated T Cell Lymphoma (EATL). Patients with EATL typically present at age 60 with lesions predominantly affecting the small bowel (especially jejunum), and less frequently the colon and stomach.1,2 There is a strong association with celiac disease, seen in at least one third of patients diagnosed with EATL.2 This patient had no known history of celiac disease and no villous atrophy was seen on EGD. EATL is aggressive and carries a poor prognosis, optimal treatment is not known.3

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