A Rare Cause of Diarrhea and Weight Loss

Abstract

Purpose: A 70-year-old man presented with a five-month history of abdominal pain, low-grade fever, fatigue, anorexia, diarrhea and a 30-pound weight loss. The initial evaluation included EGD and ileocolonoscopy, with random biopsies showing an increase in colonic intraepithelial lymphocytes and mild plasmacytosis. An abdominal CAT scan showed two separate segments of small bowel circumferential thickening. Infectious etiologies were excluded and a presumptive diagnosis of Crohn's disease was rendered. He was empirically treated with steroids with partial improvement. Several weeks later, he presented with continued symptoms, as well as diffuse edema consistent with anasarca. A repeat evaluation was negative for celiac disease, stool culture, ova and parasite, C. difficile, mycobacterium tuberculosis, HIV, and fecal fat. Laboratory showed a hemoglobin 11.1 g/dL, platelet 131 X 10ˆ9/L, BUN 32.3 mg/dL, creatinine 1.2 mg/dL, albumin 1.9 g/dL, prealbumin 29.9 mg/dL, and increased serum-feces alpha-1 antitrypsin clearance 53 (<27). These findings suggested a protein-losing enteropathy, which prompted a deep enteroscopy. Retrograde deep enteroscopy showed diffuse edema, erythema and shallow ulcerations in the distal ileum up to 80 cm from the ileocecal valve. Terminal ileum histology showed a dense, small, mature-appearing lymphocytic infiltrate expanding the lamina propria, extending into the submucosa with blunted villi without significant intraepithelial lymphocytosis. The immunophenotype was positive for CD3, CD5, CD7, CD56 and T-cell gene rearrangement, and negative for CD4, CD8, and Tdt. Enteropathy-associated T-cell lymphoma (EATL) was diagnosed. The patient was started on cyclophosphamide, etoposide, vincristine and prednisone alternating with pralatrexate. Unfortunately, he developed bacteremia, septic shock and expired six weeks later. EATL is an uncommon gastrointestinal T cell lymphoma and is strongly associated with celiac disease. Patients typically present with abdominal pain, systemic B symptoms, fatigue or anorexia. The small intestine was the most commonly involved site at diagnosis (90%), followed by the large intestine (16%) with frequent involvement of mesenteric (35%) and para-aortic or iliac lymph nodes (11%). Clinical, radiologic and endoscopic manifestations of EATL can mimic Crohn's disease, as demonstrated in this case. The development of a protein-losing enteropathy in this case broadened the differential leading to the final diagnosis. Deep enteroscopy is an important endoscopic tool to obtain tissue for the proper diagnosis of EATL, especially when suggested by other imaging modalities, such as abdominal CT scan.