Incidence and Management of Posttransplantation Lymphoproliferative Disorder After Pediatric Solid Organ Transplantation: The Norwegian Experience.

Background: Posttransplant lymphoproliferative disorder (PTLD) is a complication of solid organ transplantation (SOT) due to immunosuppression. In 2023, a pediatric PTLD register was established in Norway because of a perceived increase in the incidence of pediatric PTLD. This study aimed to analyze population-based data on the incidence and management of pediatric PTLD after SOT using the pediatric PTLD registry in Norway.

Methods: This retrospective quality assurance study collected the data of pediatric patients with PTLD after SOT in Norway from January 1, 1995, to December 31, 2023. For comparison and calculation of incidence rates, SOT patients without PTLD required a minimum of 1 year posttransplant follow-up to be included.

Results: A total of 457 patients underwent SOT (57% males) and 22 (4.8%) developed PTLD (73% males). The median age at transplant in the SOT and PTLD groups were 9.5 (interquartile range, 2.4-14.5) and 4.3 (1.1-12.5) years, respectively. Twenty patients with PTLD (91%) were Epstein-Barr virus naive at the time of transplantation. Eighteen (82%) and four (18%) patients developed early and late PTLD, respectively. Ten patients had monomorphic PTLD (45%). All patients received a reduction in immunosuppression, 15 received rituximab, and six required chemotherapy. Six patients (27%) died after PTLD, five of whom had active PTLD disease at the time of death. None of the patients experienced graft loss.

Conclusions: Our findings regarding the incidence, EBV status, sex, and age at transplantation align with those of previous studies.