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噬血细胞性淋巴组织细胞增生症:一个诊断难题。

Hemophagocytic Lymphohistiocytosis: A Diagnostic Conundrum.

作者信息

Grzybowski Brittany, Vishwanath Vijay A

机构信息

Department of Emergency Medicine, Keck School of Medicine of USC, Los Angeles, CA 90033, USA.

Department of Neurology, Division of Pediatric Neurology, Albany Medical Center, Albany, NY 12208, USA.

出版信息

J Pediatr Neurosci. 2017 Jan-Mar;12(1):55-60. doi: 10.4103/jpn.JPN_140_16.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive immune activation causing widespread inflammation and tissue destruction leading to multi-organ dysfunction and failure. Making the diagnosis of HLH could be quite challenging due to the broad range of presenting symptoms and their lack of specificity. After ruling out considerations for differential diagnoses, recognizing the most common presenting signs and symptoms of HLH, including neurologic dysfunction, and having a high clinical suspicion for HLH in the setting of inflammatory/demyelinating diseases are important for prompt diagnosis and treatment.

摘要

噬血细胞性淋巴组织细胞增生症(HLH)是一种免疫过度激活综合征,可导致广泛的炎症和组织破坏,进而引起多器官功能障碍和衰竭。由于HLH的症状表现广泛且缺乏特异性,因此其诊断颇具挑战性。在排除鉴别诊断的考虑因素后,识别HLH最常见的症状体征,包括神经功能障碍,并在炎症/脱髓鞘疾病背景下对HLH保持高度临床怀疑,对于及时诊断和治疗至关重要。

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