Clinical analysis of 11 children with hemophagocytic syndrome / 临床儿科杂志

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.