Systemic Epstein–Barr virus (EBV)-positive T-cell lymphoproliferative disorders (T-LPD) of childhood is an extremely rare disease characterized by an aggressive clinical course and very poor prognosis. We report an adolescent male with systemic EBV-positive T-LPD of childhood after primary EBV infection, resulting in a fatal clinical course within 9 days, along with autopsy findings. A 19-year-old male without an immunocompromised status presented with an acute onset of high fever, and was hospitalized for persistent fever, vomiting and diarrhea on the 5th day from onset. Laboratory data showed severe thrombocytopenia, increased ferritin level, liver dysfunction, disseminated intravascular coagulation, and anti-EBV-IgM positivity. Peripheral blood smears identified a number of atypical lymphocytes. Bone marrow aspiration revealed many atypical various-sized lymphocytes with apparent nucleoli and hemophagocytosis. Atypical lymphocytes displayed a CD8+ T-cell phenotype with monoclonal rearrangement of T-cell receptors. EBV-encoded RNA was also observed in lymphoid cells by in situ hybridization. The patient received dexamethasone and cyclosporine with no improvement, and died of tumor lysis by leukocytosis on the 9th day from onset.
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