S3064 Intestinal Post-Transplant Lymphoproliferative Disorder

Abstract

Introduction: Post-transplant lymphoproliferative disease (PTLD) is a group of lymphoid disorders arising in the setting of immunosuppression following a solid organ or stem cell transplantation. PTLD impacts 2.8% of adults and 15% of children post-orthotopic liver transplant (OLT) at a median of 8 years. The small intestine, rich in B cells, is a common site for PTLD and the treatment is chemotherapy. Here, we present a case of a patient post-OLT presenting with abdominal pain, and anemia found to have small bowel PTLD resulting in jejunal perforation with eventual resolution of symptoms after surgical resection and chemotherapy. Case Description/Methods: 65-year old obese F with non-alcoholic steatohepatitis decompensated by hepatopulmonary syndrome with O2 dependence and hepatocellular carcinoma underwent an uneventful OLT (CMV +/-) with no immediate postoperative complications and resolution of the hepatopulmonary syndrome. Presented 2 months after the OLT with worsening LLQ abdominal pain and acute anemia. An EGD and colonoscopy completed shortly after admission showed evidence of gastric, duodenal, and descending colon non-bleeding ulcers that were biopsied. Pathology showed EBV+ atypical B cell proliferation concerning for large B cell lymphoma and monomorphic-PTLD. An EBV viral load obtained at that time was elevated and subsequent CT Abdomen/Pelvis showed scattered small sub-centimeter mesenteric and retroperitoneal lymph nodes. Her immunosuppression was minimized without a change in symptoms. She subsequently developed large volume haematochezia leading to shock and underwent an EGD and colonoscopy followed by a push enteroscopy that revealed multiple ulcers but no active bleeding site. Immediately after the procedure, she developed an acute abdomen due to a perforated Jejunal ulcer. Had an emergent exploratory laparotomy and jejunectomy with subsequent improvement in her anemia. She was treated with R-CHOP for PTLD with the resolution of her presenting symptoms. Discussion: PTLD, however rare, remains the commonest post-transplant malignancy. Once there is suspicion (pain, weight loss, and anemia post OLT), EGD/Colonoscopy reveals suspicious malignant-looking ulcers, and mucosa or lymph node biopsy confirms PTLD. The patient ultimately underwent treatment with 4 cycles of R-CHOP with surveillance EBV PCR and imaging with resolution of complete symptoms. Immediate treatment should be started with lowering immune suppression and chemotherapy rather than waiting for self-resolution in PTLD cases.Figure 1.: Jejunal and Gastric ulcers (seen on prior scopes as well) were found on the Push enteroscopy just prior to the perforation of the bleeding jejunal ulcer and eventual surgical resection of the jejunum that resolved her anemia.