PTLD Involving the Colon Graft in Small Bowel Transplant Recipient

Abstract

Intestinal transplant is indicated in those who have intestinal failure with resultant life-threatening complications. Complications from intestinal transplant include surgical complications eg. graft thrombosis, rejection and complications due to a high immunosuppressive requirement eg. atypical viral/fungal infections and PTLD. We present a 23-year-old white female with combined small bowel and colonic transplant 11 months ago due to intestinal failure from small bowel volvulus. She was diagnosed with PTLD with diffuse large B-cell lymphoma 1 month ago after presenting with painless LAD. She was on tacrolimus 4 mg BID, 2000 mg/day MMF, and 5 mg/day prednisone. Her EBV DNA was > 2 million copies/ml at the time of diagnosis. She underwent reduction in immunosuppression followed by 1 cycle of R-CHOP and presented with new-onset GI bleeding. EGD to jejuno-jejunal anastomosis was unremarkable but colonoscopy showed a large amount of fresh blood in the colon and ileum. The visualization of colonic mucosa was limited but CTA showed positive luminal extravasation just beyond jejuno-jejunal anastomosis which was successfully treated with embolization. Follow-up colonoscopy to evaluate for small bowel and colonic graft rejection showed normal small bowel and colonic allograft and multiple non-bleeding discreet raised yellowish black-crusted sub-epithelial nodules obliterating nearby normal colonic vasculature in native colon. Pathology showed an atypical lymphoid infiltrate with necrosis in the native colon confirmed to be monomorphic PTLD (positive for CD79a, Pax5, Mum-1, CD 20, EBV-LMP, negative CD3, Ki-67 of 70%). Her abdominal CT scan showed progressive disease burden with extensive liver metastasis. Despite treatment, her hospital course was complicated by neutropenic fever, septic shock, malignant ascites, and spontaneous intracranial hemorrhage from CNS involvement of PTLD. Therefore, no escalation of treatment was discussed and she was made comfort care. The risk of PTLD is high in intestinal transplant recipients due to high immunosuppression requirement (tacrolimus and MMF) and occur in 15% of adults and 20% of children. Colonic involvement of PTLD may portend the aggressive nature of PTLD and be seen more in disseminated or multiple organs involvement. This case highlights a variety of symptoms of PTD in an intestinal transplant recipient from initial presentation with painless LAD followed by an obscure overt GI bleeding due to colonic PTLD