An adult case of systemic Epstein-Barr virus-positive T-cell lymphoproliferative disorder with severe hepatic dysfunction and megalosplenia

ABSTRACT

Epstein-Barr virus-positive T/NK-cell lymphoproliferative disorder (EBV+T/NK-LPD) is a continuous spectrum of diseases that share a common feature observed in T cells and NK cells: Excessive lymphoid proliferation. This disease is rare in adults and predominantly affects children with high mortality. Herein, we present a case of EBV+T-LPD that occurred in an adult with clinical manifestations of hepatic dysfunction and megalosplenia. The patient received a splenectomy at a local hospital for the treatment of megalosplenia. Before surgery, she exhibited mild hepatomegaly and normal liver function. However, after the operation, abdominal computed tomography (CT) showed obvious hepatomegaly and severely damaged liver function. After a final diagnosis of EBV+TLPD at our hospital, the patient received combination therapy with antiviral and immunosuppressive agents. At the 4-month follow-up visit, hepatic function was normal and the size of the liver decreased. Because this patient presented with hepatomegaly before the splenectomy and because hepatic dysfunction rapidly progressed after surgery, an early diagnosis of EBV+T-LPD was crucial. Splenectomy may be recommended before liver involvement to reduce negative postoperative effects on the liver (AU)

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