Early onset, EBV^- PTLD in pediatric liver-small bowel transplantation recipients: A spectrum of plasma cell neoplasms with favorable prognosis

EBV^- posttransplantation lymphoproliferative disorders (PTLDs) are rare compared with EBV⁺ PTLDs, occur later after transplantation, and have a poor response to treatment. Few studies have reported EBV ^- PTLD in pediatric solid-organ transplantation recipients. We describe 5 cases of EBV^- PTLD in recipients of combined liver and small bowel allografts ranging in age from 16 months to 7 years. EBV-PTLD developed 9-22 months (median, 15) after transplantation. Morphologically, the lesions ranged from atypical plasma cell hyperplasia (a term not currently included in the World Health Organization classification) to plasmacytoma like. In all cases, in situ hybridization for EBV was negative, and molecular studies demonstrated clonal IgH gene rearrangements. Protein electrophoresis showed multiple clonal paraproteins in 4 of 5 cases. In 2 cases with a donor-recipient sex mismatch, FISH cytogenetics demonstrated that the plasma cells were of mixed donor/recipient origin. One patient died before therapy. Four patients were treated with high-dose dexamethasone, and 1 patient subsequently required thalidomide. All 4 remain in remission 75-128 months (median, 86) after diagnosis. In contrast to reports of EBV-PTLD in adults, these plasma cell lesions occurred early after transplantation and resolved completely after minimal treatment.