Background: Posttransplant lymphoproliferative disorder is a complication of solid organ transplantation and is associated with Epstein-Barr virus. Recently, Epstein-Barr virus-related posttransplant lymphoproliferative disorder was defined as probable posttransplant lymphoproliferative disorder or proven posttransplant lymphoproliferative disorder. Probable posttransplant lymphoproliferative disorder involves significant lymphadenopathy, hepatosplenomegaly, or other end-organ manifestations, without a histological diagnosis, together with significant Epstein-Barr virus DNAemia. Proven posttransplant lymphoproliferative disorder is the detection of Epstein-Barr virus-encoded proteins in tissue specimens, together with symptoms and/or signs originating from the affected organ. Probable posttransplant lymphoproliferative disorder after pediatric liver transplantation has not been well documented. Therefore, we aimed to describe the cases of five pediatric patients with probable posttransplant lymphoproliferative disorder after liver transplantation who were successfully treated with preemptive immunosuppression reduction with or without rituximab.
Case series: All five patients (age range, 1-4 years; 2 girls and 3 boys) had Epstein-Barr virus DNAemia. Three patients developed probable posttransplant lymphoproliferative disorder within 12 months of transplantation. Three patients had significantly high Epstein-Barr viral loads, but the other two patients with lymphadenopathy and end-organ manifestation had relatively low Epstein-Barr viral loads. Early-onset pediatric posttransplant lymphoproliferative disorder with significant Epstein-Barr virus DNAemia is almost universally Epstein-Barr virus-related. Biopsy was not performed due to the relative inaccessibility of the lesions and young age of the patients.
Conclusions: If the patient’s symptoms are too mild, excisional biopsy is too difficult to perform, or the patient is too sick to undergo invasive procedures, initiating preemptive treatment without a histological diagnosis could be the treatment option.