RESUMO
Epstein-Barr Virus (EBV) is a ubiquitous herpes type virus that is associated with post-transplant lymphoproliferative disorder (PTLD). Usual management includes reduction or cessation of immunosuppression and in some cases chemotherapy including rituximab. However, limited therapies are available if PTLD is refractory to rituximab. Several clinical trials have investigated the use of EBV-directed T cells in rituximab-refractory patients; however, data regarding response is scarce and inconclusive. Herein, we describe a patient with EBV-PTLD refractory to rituximab after orthotopic heart transplantation (OHT) requiring EBV-directed T-cell therapy. This article aims to highlight the unique and aggressive clinical presentation and progression of PTLD with utilization of EBV-directed T-cell therapy for management and associated pitfalls.
Assuntos
Infecções por Vírus Epstein-Barr , Transplante de Coração , Transplante de Células-Tronco Hematopoéticas , Transtornos Linfoproliferativos , Humanos , Pré-Escolar , Herpesvirus Humano 4 , Rituximab/uso terapêutico , Infecções por Vírus Epstein-Barr/terapia , Infecções por Vírus Epstein-Barr/tratamento farmacológico , Transtornos Linfoproliferativos/diagnóstico , Transtornos Linfoproliferativos/etiologia , Transtornos Linfoproliferativos/terapia , Terapia Baseada em Transplante de Células e TecidosRESUMO
Truncus arteriosus is an extremely rare and complex form of congenital heart disease. Surgical strategies vary depending on anatomic subtype. In this tutorial, we present our surgical technique for single stage repair of a truncus arteriosus with interrupted aortic arch (Van Praagh type A4).
