Clinical analysis of 11 children with hemophagocytic syndrome
10.3969/j.issn.1000-3606.2015.07.013
- VernacularTitle:儿童噬血细胞综合征11例临床分析
- Author:
Xinbao WANG
- Publication Type:Journal Article
- Keywords:
hemophagocytic syndrome;
Epstein-Barr virus;
child
- From:
Journal of Clinical Pediatrics
2015;(7):655-657
- CountryChina
- Language:Chinese
-
Abstract:
ObjectivesTo explore the clinical presentation, diagnosis and treatment of hemophagocytic syndrome (HLH) in children.MethodsThe clinical data from 11 hospitalized pediatric patients with HLH were collected and retrospectively analyzed from 2009 to 2013.ResultsIn 11 pediatric patients with HLH, 6 patients were Epstein-Barr virus associated-HLH, 1 patient was T-cell lymphoma associated-HLH, 2 patients had unknown reason, 2 patients had mutations in the UNC13D gene coding sequence, c.2459C>T/p.A832V (alanine to valine mutation) and c.3067C>T/p.R1023C (arginine to cysteine mutation) respectively. In 11 patients, 6 patients were improved after treatment and 5 patients were died.ConclusionsThe HLH in chil-dren lacks speciifc clinical presentation and progresses rapidly. It should be diagnosed and treated in time.
