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成人斯蒂尔病合并心肌炎和噬血细胞性淋巴组织细胞增生症:罕见表现及致命结局

Adult-onset Still's disease with myocarditis and hemophagocytic lymphohistiocytosis: Rare manifestation with fatal outcome.

作者信息

Gupta Devika, Jagani Rajat, Mendonca Satish, Rathi Khushi Ram

机构信息

Department of Pathology and Laboratory Science, Armed Forces Medical College, Command Hospital, Pune, Maharashtra, India.

出版信息

Indian J Pathol Microbiol. 2016 Jan-Mar;59(1):84-6. doi: 10.4103/0377-4929.174825.

Abstract

Adult-onset Still's disease (AOSD) is a rare inflammatory disorder of unknown etiology characterized by fever, evanescent pink salmon rash, arthritis, and multiorgan involvement. Here, we report an unusual manifestation of AOSD in a 40-year-old male who presented to our hospital with pyrexia of unknown origin and rash of 3 weeks duration. All his serological investigations and imaging studies were unremarkable. He was fulfilling clinical and laboratory criteria as per Yamaguchi for AOSD and was managed for the same. Our patient did not respond well to the treatment, had a downhill course, and succumbed to his illness. Autopsy confirmed myocarditis and florid bone marrow reactive hemophagocytosis as the cause of his death.

摘要

成人斯蒂尔病(AOSD)是一种病因不明的罕见炎症性疾病,其特征为发热、一过性粉红色鲑鱼皮疹、关节炎和多器官受累。在此,我们报告一例40岁男性AOSD的不寻常表现,该患者因不明原因发热和持续3周的皮疹前来我院就诊。他所有的血清学检查和影像学检查均无异常。他符合山口标准中AOSD的临床和实验室标准,并接受了相应治疗。我们的患者对治疗反应不佳,病情逐渐恶化,最终因病死亡。尸检证实心肌炎和显著的骨髓反应性噬血细胞增多是其死亡原因。

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