川崎病并发巨噬细胞活化综合征(附3例报告并文献分析)

目的了解川崎病并发巨噬细胞活化综合征(MAS)病儿的特点。方法总结我院2009年11月—2010年4月收治的3例川崎病并发MAS病儿的临床特点,并对文献报道17例川崎病并发MAS病儿进行分析。结果 20例川崎病并发MAS病儿,男14例,女6例;年龄为1~11岁,平均(5.35±3.66)岁;临床特征主要有持续发热、肝脾大、血细胞减少、凝血功能障碍、肝酶升高、高三酰甘油血症、低纤维蛋白原血症、血清铁蛋白增高,骨髓细胞学检查找到噬血细胞。联合应用糖皮质激素、大剂量丙种球蛋白及免疫抑制剂治疗有效,20例病儿除1例放弃治疗,其余均存活。结论 MAS是川崎病的一种严重并发症,病情凶险,早期合理的治疗可改善川崎病并发MAS病儿的预后。

KAWASAKI DISEASE FOLLOWED BY MACROPHAGE ACTIVATION SYNDROME: REPORT OF THREE CASES AND REVIEW OF LITERATURES

Objective To understand clinical characteristics of Kawasaki disease followed by macrophage activation syndrome(MAS) in children. Methods Clinical features of three cases of Kawasaki disease associated with MAS treated in our hospital,from November 2009 to April 2010,were summarized,and 17 cases reported in literature were analyzed as well. ResultsThere were 14 boys and six girls in this group,age ranged(mean) 1-11(5.35±3.66) years old.The main clinical manifestations were: persistent fever,hepatosplenomegaly,hypocytosis,coagulation disorders,hepatic enzymes increased,hypertriglyceridemia,hypofibrinogenemia,serum ferritin increased;hemophagocytes found in bone marrow.Co-therapy with hormonal,high-dose gamma globulin,and immunosuppressive agents was effective for this disease.Of the 20 cases reviewed,one gave up treatment,the rest 19 children survived. Conclusion MAS is a serious complication of Kawaski disease,an early reasonable therapy may improve the prognosis of children with this condition.