A 13-Year-Old Boy with Post-transplantation Lymphoproliferative Disorder Presenting with Obscure GI Bleeding

Abstract

Purpose: Post-transplantation lymphoproliferative disorders (PTLD) are well recognized complications of chronic immunosuppression in organ transplant recipients, accounting for 21% of all malignancies in transplant recipients compared to 5% in the general population. The diagnosis of PTLD is often difficult, due to the varied manifestations resulting in a delay in diagnosis. We report an unusual presentation of PTLD in a pediatric patient where the diagnosis was reached only after extensive investigation. Case Report: This is a 13-year-old boy with a history of cystic fibrosis who is status post deceased donor EnBloc liver and pancreas transplantation. His immune suppression regimen included tacrolimus 1.5 mg bid. Two years after his transplant, this patient presented with melena with a hemoglobin of 4.6 g/dL. After adequate resuscitation, he underwent an upper endoscopy, which showed multiple small prepyloric and duodenal ulcerations without signs of recent hemorrhage. Biopsies of these ulcers showed acute inflammation. Capsule endoscopy revealed scattered duodenal erosions and two kissing erosions in the distal duodenum, without stigmata of recent bleed. Despite being on proton pump inhibitors, the patient continued to have melena, necessitating transfusions every few days. A second upper endoscopy was performed, which showed portal hypertensive gastropathy, a gastric varix, and complete healing of prior ulcers. Octreotide was initiated. A tagged RBC scan revealed a potential bleeding source “near the anastomosis of the native to transplanted duodenum or proximal jejunum.” A visceral arteriogram was negative. An enteroscopy was performed. It revealed no signs of recent bleed within the suspected proximal hepatic limb of the Roux. However, a 10 mm clean-base, friable ulcer was present in the jejunum, with significant oozing when biopsied. The pathologic diagnosis of this ulcer was “Monomorphic B-cell PTLD, involving small intestinal mucosa.” Conclusion: PTLD are rare but potentially fatal complications of solid organ transplantation. They often affect the gastrointestinal (GI) tract, lungs, liver, central nervous system, and the allograft itself. Clinical manifestations range from benign polyclonal lymphoproliferation (infectious mononucleosis-type acute illness) to aggressive and disseminated malignant disease. The incidence of PTLD increases with the intensity of induction or rescue immunosuppression, and particularly following monoclonal or polyclonal anti-lymphocyte therapy. GI features typically include abdominal pain, fever, and bowel perforation. Although GI bleeding is a less common presentation, PTLD should be considered in transplant receipts presenting with unexplained GI hemorrhage.