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.
