儿童噬血性淋巴组织细胞增生症的诊断和治疗进展

Diagnosis and therapy of children hemophagocytic lymphohistiocytosis

噬血性淋巴组织细胞增生症(hemophagocytic lymphohistiocytosis,HLH)是一种病情进展迅速,可危及生命的炎症反应综合征,在儿童发病率及病死率较高。其病因和临床表现多样,诊断较困难,挽救治疗方案国内外尚未统一。该文对HLH的类型、病理机制、临床表现、实验室检查、诊断及治疗作一综述,为该病的及时诊断和个体化治疗提供参考。

Hemophagocytic lymphohistiocytosis(HLH)is a life-threatening inflammatory response syndrome,which progresses rapidly.Its etiology and clinical manifestations are diverse,and its diagnosis is difficult,and the rescue treatment has not been unified at home and abrord.The paper reviews the classification,pathogenesis,clinical manifestations,laboratory indexs,diagnosis and treatment of HLH,in order to provide reference for timely diagnosis and individualized treatment.