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重症发热伴血小板减少综合征致死病例中的噬血细胞性淋巴组织细胞增生症

Hemophagocytic Lymphohistiocytosis in a Fatal Case of Severe Fever with Thrombocytopenia Syndrome.

作者信息

Nakano Ayako, Ogawa Hirohisa, Nakanishi Yoshinori, Fujita Hiromi, Mahara Fumihiko, Shiogama Kazuya, Tsutsumi Yutaka, Takeichi Toshiaki

机构信息

Department of Internal Medicine, Local Incorporated Administrative Agency Tokushima Prefecture Naruto Hospital, Japan.

Department of Pathology and Laboratory Medicine, Tokushima University Graduate School of Medicine, Japan.

出版信息

Intern Med. 2017;56(12):1597-1602. doi: 10.2169/internalmedicine.56.6904. Epub 2017 Jun 15.

Abstract

Severe fever with thrombocytopenia syndrome (SFTS) is an emerging disease caused by a novel Bunyavirus with a high mortality rate. We herein report a fatal case of an 86-year-old woman with SFTS complaining of a fever, fatigue, and bicytopenia. Her condition deteriorated with rapid progression of bleeding tendency, disturbance of consciousness, and multiple organ failure leading to death on Day 6 of her illness. The histopathological findings in the autopsy revealed marked infiltration of macrophages with hemophagocytosis in the bone marrow, liver, and spleen leading to a diagnosis of hemophagocytic lymphohistiocytosis (HLH). HLH might be a critical pathogenesis in fatal cases of SFTS.

摘要

发热伴血小板减少综合征(SFTS)是一种由新型布尼亚病毒引起的新发疾病,死亡率很高。我们在此报告一例86岁女性SFTS致死病例,该患者主诉发热、乏力和血细胞减少。她的病情恶化,出血倾向迅速进展、意识障碍和多器官功能衰竭,在患病第6天死亡。尸检的组织病理学结果显示,骨髓、肝脏和脾脏中有大量巨噬细胞浸润并伴有噬血细胞现象,从而诊断为噬血细胞性淋巴组织细胞增生症(HLH)。HLH可能是SFTS致死病例的关键发病机制。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fda2/5505921/44c44a01fbd9/1349-7235-56-1597-g001.jpg

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