Systemic epstein-barr virus-positive t-cell lymphoproliferative disease of childhood

Systemic Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disease of childhood is a rare, aggressive T-cell malignancy recently recognized in the 2008 World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues. This disease classically arises in the context of primary acute or chronic active EBV infection, and it is characterized by a clonal proliferation of EBV-infected T cells with an activated cytotoxic phenotype. Most patients have a fulminant clinical course. The clinical and pathologic features of the disease overlap with other conditions, such as infectious mononucleosis, chronic active EBV infection, hemophagocytic lymphohistiocytosis, and natural killer cell malignancies. Here we report a case of systemic EBV+ T-cell lymphoproliferative disease of childhood, and the key features and diagnostic pitfalls of this entity are discussed.