Yu Binfeng, Zhu Yongfen, Jin Zhihua, Han Fei, Lv Fangfang
Department of Liver and Infectious Diseases, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, 310000, China.
Department of Liver and Infectious Diseases, Shaoxing Central Hospital, Shaoxing, China.
Virol J. 2025 Feb 15;22(1):39. doi: 10.1186/s12985-025-02649-2.
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.
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.
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.
血小板减少是戊型肝炎病毒（HEV）感染常见的肝外表现，通常为一过性且自限性。血栓性血小板减少性紫癜（TTP）是一种罕见且致命的血液疾病，其特征为微血管病性溶血性贫血、严重血小板减少及器官受累。HEV感染与TTP之间的联系仍不明确。
一名74岁女性因发热、乏力、恶心及黄疸10天转诊至我院。肝功能异常、HEV IgM和IgG阳性以及HEV-RNA病毒血症提示急性戊型肝炎诊断成立，随后血小板计数急剧下降。外周血涂片出现裂体细胞，同时ADAMTS13活性降低，强烈提示TTP诊断。采用包括2个疗程淋巴细胞血浆置换（LPE）、4个疗程治疗性血浆置换、糖皮质激素及利妥昔单抗的联合治疗，促使血小板恢复。随访期间未观察到TTP复发。迄今为止，这是首例在HEV病毒血症病程中发生TTP首发事件的患者。同时，LPE首次用于治疗HEV相关TTP。
该病例突出了对新发生严重血小板减少的戊型肝炎患者排除TTP的必要性，以及LPE加利妥昔单抗治疗此类患者的价值。
