化疗治疗成人斯蒂尔病继发巨噬细胞活化综合征导致休克3例并文献复习

Chemotherapy for 3 cases of shock caused by macrophage activation syndrome secondary to adult-onset Still’s disease and an associated literature review

成人斯蒂尔病(adult-onset Still’s disease,AOSD)合并巨噬细胞综合征(macrophage activation syndrome,MAS)是一类少见但严重的自身免疫性疾病,其病死率高达30%~50%。多数文献报道AOSD继发MAS可导致血细胞减少及肝、肾、肺等多器官功能损害,但AOSD继发MAS导致危及生命的循环衰竭且常规抗休克治疗无效的情况却鲜有报道。本研究对安徽医科大学第二附属医院重症医学科于2017年10月至2018年4月收治的3例AOSD继发MAS导致休克的患者的临床资料进行报告,并复习相关文献资料。研究发现临床上一些血液系统肿瘤或风湿性疾病患者出现难以解释的循环衰竭,特别是合并铁蛋白显著升高时,往往提示可能存在噬血细胞性淋巴组织细胞增生症(hemophagocytic lymphohistiocytosis,HLH)或MAS,而早期识别与及时治疗有利于改善患者预后。

Adult-onset Still’s disease(AOSD)with macrophage activation syndrome(MAS)is a rare autoimmune disease with a reported fatality rate as high as 20%~30%.Many studies have reported that MAS secondary to AOSD can lead to hemocytopenia and damage the liver,kidney,lung and other organs,but there have been few reports of life-threatening circulatory failure where circulation is ineffective and difficult to treat by conventional therapy.This study reports the clinical data of 3 patients with shock caused by MAS secondary to AOSD admitted to the Intensive Care unit(ICU)from October 2017 to April 2018,and then presents a review of the relevant literature.This case series shows that circulatory failure in some patients with hematological malignancies or rheumatic diseases that is difficult to explain by conventional theories,especially when accompanied by significant increase in ferritin levels,often indicates the possible existence of hemophagocytic lymphohistiocytosis or MAS.Early recognition and timely treatment may be beneficial to improve patients’prognosis.