DEP方案联合鞘注治疗儿童噬血细胞综合征中枢神经系统重症受累一例

DEP regimen combined with intrathecal injection in the treatment of severe central nervous system involvement in a child with hemophagocytic lymphohistiocytosis and literature review

目的提高对噬血细胞综合征合并中枢神经系统受累(CNS-HLH)临床表现的认识,做到及时诊断、积极治疗以期改善患者预后。方法分析1例重症噬血细胞综合征合并中枢神经系统受累患儿的临床资料,包括临床特点、诊疗经过及随访结果。结果及时作出CNS-HLH明确诊断,尽早给予积极治疗,减少神经系统后遗症风险,改善患者预后。结论CNS-HLH临床表现具有多样化,及时诊断需借助MRI等检测手段,一旦诊断明确,DEP方案联合鞘内注射甲氨蝶呤、地塞米松可明显改善预后。

Objective To improve the understanding of the clinical manifestations of hemophagocytic lymphohistiocytosis combined with central nervous system involvement(CNS-HLH),so as to achieve timely diagnosis and active treatment in order to improve the prognosis of patients.Methods The clinical data of a patient with severe hemophagocytic lymphohistiocytosis complicated by central nervous system involvement were analyzed,including clinical features,diagnosis and treatment process and follow-up results.Results A clear diagnosis of CNS-HLH was made in time,and active treatment was given as soon as possible to reduce the risk of neurological sequelae and improve the prognosis of patients.Detection methods are needed for timely diagnosis.Once the diagnosis is clear,DEP regimen combined with intrathecal injection of methotrexate and dexamethasone can significantly improve the prognosis.