Abstract
BACKGROUND: Thrombocytopenia is a common extrahepatic manifestation of hepatitis E virus (HEV) infection and is usually transient and self-limited. Thrombotic thrombocytopenic purpura (TTP) is a rare and lethal blood disorder characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and organ involvement. The link between HEV infection and TTP is still unclear. CASE PRESENTATION: A 74-year-old female was referred to our hospital with complaints of fever, fatigue, nausea and jaundice for 10 days. Liver dysfunction, positive IgM and IgG of HEV, and HEV-RNA viremia prompted the diagnosis of acute hepatitis E, which was followed by a dramatic decline in the platelet count. The presence of schistocytes in the peripheral blood smear, along with decreased ADAMTS13 activity, strongly suggested a diagnosis of TTP. Combination therapy, including 2 courses of lymphoplasmapheresis (LPE), 4 courses of therapeutic plasma exchange, glucocorticoids and rituximab, was applied and contributed to the recovery of platelet. No recurrence of TTP was observed during the follow-up period. To date, this is first patient who developed the initial episode of TTP during the course of HEV viremia. In the meanwhile, LPE was used for the first time in the treatment of HEV-associated TTP. CONCLUSIONS: This case highlights the necessity of ruling out TTP in hepatitis E patients with newly developed and severe thrombocytopenia and the values of LPE plus rituximab in treating such patients.