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类似于急性坏死性脑病的脑损伤作为噬血细胞性淋巴组织细胞增生症的一种特殊表现——由超敏反应引起。

Brain damage resembling acute necrotizing encephalopathy as a specific manifestation of haemophagocytic lymphohistiocytosis - induced by hypersensitivity.

作者信息

Dai Dongling, Wen Feiqiu, Liu Sixi, Zhou Shaoming

机构信息

First Affiliated Hospital of Jinan University, No.601 Huangpu Avenue West, Guangzhou, 510630, China.

Shenzhen Children's Hospital, 7019, Yitian Road, Shenzhen, 518036, Futian District, China.

出版信息

Ital J Pediatr. 2016 Aug 31;42(1):79. doi: 10.1186/s13052-016-0286-z.

Abstract

BACKGROUND

Both haemophagocytic lymphohistiocytosis and acute necrotizing encephalopathy are life-threatening condition. It presents major diagnostic difficulties, since it may have a diversity in clinical picture and with many conditions leading to the same clinical presentation. So it is key important to understand the disorders.

CASE PRESENTATION

We report a pediatric case of haemophagocytic lymphohistiocytosis with specific presentation which predominantly featured as acute necrotizing encephalopathy of childhood. We discuss the diagnosis and differential diagnosis, and speculate the etiology of haemophagocytic lymphohistiocytosis is due to hypersensitivity.

CONCLUSION

Haemophagocytic lymphohistiocytosis and brain damage in this case may be induced by hypersensitivity, which have good clinical outcome if diagnosed and treated early.

摘要

背景

噬血细胞性淋巴组织细胞增生症和急性坏死性脑病均为危及生命的病症。其诊断存在重大困难,因为临床表现可能多样,且多种病症可导致相同的临床表现。因此,了解这些病症至关重要。

病例报告

我们报告一例具有特殊表现的儿童噬血细胞性淋巴组织细胞增生症病例,其主要特征为儿童急性坏死性脑病。我们讨论了诊断和鉴别诊断,并推测噬血细胞性淋巴组织细胞增生症的病因是超敏反应。

结论

该病例中的噬血细胞性淋巴组织细胞增生症和脑损伤可能由超敏反应诱发,若早期诊断并治疗,临床预后良好。

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