Systemic EBV-Positive T-Cell Lymphoma of Childhood

**Semantic type:** Neoplastic Process

**Definition:** An aggressive and life-threatening, EBV-positive T-cell lymphoma affecting children. It is more prevalent in Taiwan and Japan. Clinically, it presents with acute onset of fever and generalized malaise, followed by hepatosplenomegaly and liver failure. Morphologically it is characterized by the presence of infiltrating T-lymphocytes which are usually small and erythrophagocytosis. Most patients have a fulminant clinical course.

**Synonyms:** - Systemic EBV-Positive T-Cell Lymphoproliferative Disease of Childhood - Systemic EBV-Positive T-Cell Lymphoproliferative Disorder of Childhood - Systemic Epstein-Barr Virus-Positive T-Cell Lymphoma of Childhood