The characteristics of PTLD differ between pediatric and adult recipients. ![]() 4, 5 PTLD encompasses a heterogeneous group of diseases that inherently have a wide range of clinicopathological characteristics, from incidental asymptomatic findings to fulminant lymphoma presenting with multiorgan failure. However, during the past 10 to 15 years, the incidence of PTLD has increased, which has been associated with high morbidity and mortality after SOT and HSCT. 3 Since it was first described in 1969 by Penn et al., PTLD was believed to be a relatively rare disorder. Posttransplant lymphoproliferative disorder (PTLD) is one of the most common malignancies occurring after solid organ transplantation (SOT) and hematopoietic stem cell transplantation (HSCT). Long-term complications such as metabolic disorders due to immunosuppressive medications, chronic rejection, and de novo cancers need to be solved. However, problems exist beyond short-term complications such as hepatic artery thrombosis, portal vein thrombosis, or infection. There has been significant improvement in terms of the outcomes for patients and graft survival in recent decades. Liver transplantation is the only viable therapeutic option for children with end-stage liver disease. If the patient’s symptoms are too mild, if excisional biopsy is too difficult to perform, or if the patient is too sick to undergo an invasive procedure, initiating preemptive treatment without a histological diagnosis could be the treatment option. Biopsy was not performed in any patient due to the relative inaccessibility of the lesion and young age of the patients. ![]() Early onset pediatric PTLD with significant EBV DNAemia is almost universally EBV-related. Further, three patients had a significantly high EBV viral load, but the other two patients with lymphadenopathy and end-organ manifestation had a relatively low EBV viral load. Three patients developed probable PTLD within 12 months of transplantation. All five patients (age range, 1–4 years two girls and three boys) had EBV DNAemia. Therefore, here, we aimed to describe cases of five pediatric patients with probable PTLD after liver transplantation, who were successfully treated with preemptive immunosuppression reduction with or without rituximab. Probable PTLD after pediatric liver transplantation has not been well documented. Proven PTLD is the detection of EBV-encoded proteins in a tissue specimen, together with symptoms and/or signs originating from the affected organ. Probable PTLD involves significant lymphadenopathy, hepatosplenomegaly, or other end-organ manifestations, without a histological diagnosis, together with significant EBV DNAemia. ![]() Recently, EBV-related PTLD was defined as probable PTLD or proven PTLD. Posttransplant lymphoproliferative disorder (PTLD) is a complication of solid organ transplantation and is associated with Epstein-Barr virus (EBV).
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