儿童噬血细胞淋巴组织细胞增生症治疗进展  

Advances in childhood hemophagocytic iymphohistiocytosis therapies

作　　者：张坤[1] 王弘[1] Zhang Kun;Wang Hong(Hematological Treatment Center, Pediatric Hematological Department, Shengjing Hospital of China Medical University, Shenyang 110022, China)

机构地区：[1]中国医科大学附属盛京医院血液病治疗中心小儿血液科,沈阳110022

出　　处：《国际儿科学杂志》2018年第4期299-302,共4页International Journal of Pediatrics

摘　　要：噬血细胞淋巴组织细胞增生症（hemophagocytic lymphohistiocytosis，HLH）在任何年龄均可发病，儿童所占比例高达80%，并且病死率较高。研究发现以依托泊苷和地塞米松为主的HLH-04和HLH-94方案是快速终止炎症风暴有效方法，此外脂质体阿霉素、抗胸腺细胞球蛋白、血浆置换也有一定疗效。针对EB病毒相关性HLH应早期应用依托泊苷，必要时加入针对干扰素-γ、T细胞、B细胞的靶向药物治疗；淋巴瘤相关性HLH则以CHOP或COP方案化疗为主；免疫相关性HLH在大剂量激素、丙种球蛋白的基础上联合血浆置换，并合理使用单克隆靶向药物效果良好；对于家族性HLH、难治性HLH在疾病缓解期行异基因造血干细胞移植是唯一的治疗方法。Although hemophagocytic lymphohistiocytosis（HLH） may occur at any age with high mor- tality, actually 80% of the patients were diagnosed in childhood. Studies have shown that HLH-04/HLH-94 pro- tocol based on etoposide and dexamethasone therapies have effects for rapid termination of the inflammatory storm. Moreover, the administration of liposomal doxorubicin, anti-thymocyte globulin and plasma exchange also showed certain effects. Etoposide should be applied as soon as possible for Epstein-Barr virus HLH, in some ca- ses targeted drugs for interferon-γ、Tor B cells were also needed. Lymphoma associated HLH mainly depended on chemotherapy with CHOP or COP protocol. High-dose corticosteroid combined with intravenous irnmuno- globulin and plasma exchange showed good treatment for immune-associated HLH. Furthermore, the rational uses of monoclonal targeted drugs could be better. Allogeneic hematopoietic stem cell transplantation was the only way for familial HLH or refractory HLH in the remission stage.

关 键 词：噬血细胞淋巴组织细胞增生症 儿童 治疗 

分 类 号：R725.5[医药卫生—儿科]