小児心臓移植後の移植後リンパ増殖性疾患（PTLD）

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Post-transplant lymphoproliferative disorders (PTLD) remain a significant cause of morbidity and mortality after pediatric heart transplantation. More than 80 % of cases are of B-cell origin and are positive for Epstein-Barr virus (EBV). The pathogenesis of EBV-positive PTLD appears to be correlated with the uncontrolled proliferation of latently EBV-infected B cells arising from a lack of EBV-specific cytotoxic T-lymphocyte function. PTLD includes a spectrum of diseases ranging from reactive lymphoid proliferation to malignant lymphoma. The risk factors underlying the development of PTLD include the degree of immunosuppression, the EBV serostatus of the recipient, the time since transplantation, and the recipient's age and ethnicity. In the pediatric age group, most PTLD cases occur in EBV-negative recipients of EBV-positive donor organs. As part of routine evaluations of patients with a high risk of PTLD, EBV monitoring is useful for the early detection of this complication. A high index of suspicion is key to an early and accurate diagnosis of PTLD. The subsequent therapeutic goals are the eradication of PTLD and the preservation of graft function. The treatment outcomes for PTLD have steadily improved over the past decade, partly because of the availability of rituximab and everolimus.