RESUMEN
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.
Asunto(s)
Infecciones por Virus de Epstein-Barr , Trasplante de Corazón , Trasplante de Células Madre Hematopoyéticas , Trastornos Linfoproliferativos , Humanos , Preescolar , Herpesvirus Humano 4 , Rituximab/uso terapéutico , Infecciones por Virus de Epstein-Barr/terapia , Infecciones por Virus de Epstein-Barr/tratamiento farmacológico , Trastornos Linfoproliferativos/diagnóstico , Trastornos Linfoproliferativos/etiología , Trastornos Linfoproliferativos/terapia , Tratamiento Basado en Trasplante de Células y TejidosRESUMEN
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).