Kaneko Masahiko, Azuma Taichi, Yasukawa Masaki, Shinomiya Hiroto
Kansenshogaku Zasshi. 2015 Sep;89(5):592-6. doi: 10.11150/kansenshogakuzasshi.89.592.
Severe fever with thrombocytopenia syndrome (SFTS) is a recently identified emerging viral infectious disease in China that is caused by a novel phlebovirus in the family Bunyaviridae, SFTS virus, with an average case fatality rate of 12-30%. A cytokine storm with abnormally expressed cytokine profiles is associated with the disease severity. Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and lifethreatening syndrome associated with excessive immune activation. We report herein on a fatal case of SFTS complicated by HLH. Consecutive plasma exchange and immunomodulatory therapy was ineffective in our case. The pathognomonic histological feature was necrotizing lymphadenitis with massive hemophagocytosis of systemic lymphoid tissues with SFTS viruses and SFTS-RNA copies. No specific treatment of SFTS is available, and an effective treatment strategy for patients with rapidly progressing SFTS has not been established. Appropriate immunomodulatory therapy is necessary for SFTS patients complicated by HLH.
发热伴血小板减少综合征（SFTS）是中国最近发现的一种新发病毒性传染病，由布尼亚病毒科的一种新型静脉病毒——SFTS病毒引起，平均病死率为12% - 30%。细胞因子谱异常表达的细胞因子风暴与疾病严重程度相关。噬血细胞性淋巴组织细胞增生症（HLH）是一种与过度免疫激活相关的侵袭性且危及生命的综合征。我们在此报告一例并发HLH的SFTS致死病例。在我们的病例中，连续血浆置换和免疫调节治疗均无效。特征性组织学表现为坏死性淋巴结炎，全身淋巴组织出现大量吞噬SFTS病毒和SFTS - RNA拷贝的噬血细胞现象。目前尚无针对SFTS的特异性治疗方法，且尚未建立针对快速进展性SFTS患者的有效治疗策略。对于并发HLH的SFTS患者，适当的免疫调节治疗是必要的。
