Systemic Epstein-Barr virus-positive T cell Iymphoproliferative disease of childhood is a recently described, potentially lethal clinical entity in children and young adults, characterized by a clonal proliferation of EBV-infected T-cells with an activated cytotoxic phenotype. It is typically prevalent in Asian countries and Mexico, and its incidence is unknown in our country. This report describes a case of systemic Epstein-Barr virus positive T-cell lymphoproliferative disease of childhood with idle progression and fatal outcome.
