Recurrent Intestinal Perforation After Cessation of Immunosuppression in Posttransplant Lymphoproliferative Disease in Pediatric Liver Transplant Recipient

Posttransplant lymphoproliferative disease (PTLD) is the most common malignant complication after solid organ transplantation. Gastrointestinal involvement as the presentation in early PTLD can occur in 25–30% of pediatric liver transplant recipients and can be the only system involved in 20%. Recurrent gastrointestinal perforation due to resolution of PTLD is an extremely rare complication. We report a 13-month-old male child diagnosed with PTLD, treatment of which lead to recurrent intestinal perforations. The child presented with gastrointestinal bleed 5 months after living donor liver transplantation for biliary atresia. Evaluation was suggestive of PTLD and biopsy confirmed diffuse large B-cell lymphoma. Positron emission tomography scan showed diffuse involvement of small intestine and ileum. Tacrolimus was withdrawn abruptly following diagnosis of PTLD as there was associated renal impairment. Child developed six episodes of small intestinal perforations over 3 weeks which required multiple laparotomies with closure of perforation and/or small bowel resection. Complete remission was achieved six months after diagnosis with cessation of immunosuppression alone and child is alive at 48 months follow-up without any recurrence. To avoid bowel perforation and complications related to tumor necrosis, immunosuppression reduction in PTLD should be gradual while carefully monitoring Epstein–Barr virus levels, tumor response, graft function, and general health status of the patient.