Majumder Anusree, Sen Debraj
Armed Forces Medical College and Command Hospital (Southern Command), Department of Pathology and Laboratory Sciences, Pune, India.
Armed Forces Medical College and Command Hospital (Southern Command), Department of Radiodiagnosis and Imaging, Pune, India.
Autops Case Rep. 2021 Apr 15;11:e2021243. doi: 10.4322/acr.2021.243.
Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal syndrome resulting from a hyperactivated immune system. Diverse patient profiles and clinical presentations often result in misdiagnosis. This article describes the varied clinical presentations and autopsy findings in three patients with this entity. The etiopathogenesis of HLH, its disparate and confounding clinical features, the diagnostic criteria, and management principles are also briefly reviewed.
噬血细胞性淋巴组织细胞增生症（HLH）是一种由免疫系统过度激活引起的罕见且可能致命的综合征。不同的患者特征和临床表现常常导致误诊。本文描述了三名患有该疾病的患者的各种临床表现和尸检结果。HLH的病因发病机制、其不同且令人困惑的临床特征、诊断标准及治疗原则也进行了简要综述。
