儿童嗜酸性粒细胞增多综合征并多发动静脉血栓形成1例并文献复习  

Hypereosinophilic syndrome in children with pulmonary embolism and venous thrombosis:a case report and literature review

作　　者：王勇[1] 林明星[1] 林晓霞[1] 陈燕惠[1] 李健[2] WANG Yong;LIN Mingxing;LIN Xiaoxia;CHEN Yanhui;LI Jian(Department of Pediatrics, 2. Department of Hematologyin Children Union Hospital, Fujian Medical University, Fuzhou 35001, China)

机构地区：[1]福建医科大学附属协和医院小儿内科,福州350001 [2]福建医科大学附属协和医院小儿血液科,福州350001

出　　处：《中国小儿血液与肿瘤杂志》2018年第3期137-142,共6页Journal of China Pediatric Blood and Cancer

基　　金：福建省血液医学中心建设项目[闽政办(2017)4号]

摘　　要：目的提高对儿童嗜酸性粒细胞增多综合征(HES)并血小板减少及多发动静脉血栓的认识。方法回顾性分析1例HES并血小板减少、肺栓塞及下肢深静脉血栓患儿的病例资料,并复习相关文献,总结其临床特征及诊治经验。结果患儿,男,12岁,临床表现包括皮疹、发热、胸痛、下肢肿胀及破溃;辅助检查:血常规提示反复嗜酸性粒细胞增多及血小板减少,D-二聚体升高,3P+FDPP阳性,双下肢血管彩超提示深静脉多发血栓形成,肺动脉CT提示肺栓塞;予短程糖皮质激素联合静脉注射用人血免疫球蛋白治疗,嗜酸性粒细胞及血小板虽可恢复正常,但易反复;予低分子肝素钠抗凝2个月,血栓进展;维持抗凝同时口服强的松2个月,症状消失、血常规恢复正常、血栓完全吸收。结论 HES本身可引起血小板低下;HES合并D二聚体升高时,需警惕深静脉血栓形成及脏器栓塞,尽早抗凝治疗联合糖皮质激素治疗成功率高,可减少严重血栓性事件发生。Objective To discuss the clinical features of hypereosinophilic syndrome （HES） in children with pulmonary embolism and venous thrombosis and raise awareness of this disease. Methods The clinical datas of a child with HES complicated with pulmonary embolism and venous thrombosis were analyzed restrospec-tively. The related literatures were reviewed. The patients＇ clinical manifestations and the experience were summarized. Results A previously healthy 12 years-old boy presented with rash, fever, cough, lower extremity swelling and skin ulcers. Laboratory examinations showed that persistent eosinophilia and repeated thromboeytopenia, D-dimer increased, and 3P and FDP test positive. Pulmonary artery CT-scanning demonstrated pulmonary embolism and color Doppler ultrasound prompted multiple deep vein multiple thrombosis. At first the patient was provided with therapy of short-term high- dose glucocorticoid and intravenous gamma globulin and his condition was getting better once. However, eosinophilia and sodium was used for 2 months, thrombocytopenia reappeared after a few days. Then low- molecular-weight heparinbut thrombosis was no significant change. At last, anticoagulation while oral prednisone treatment was taken. Two month later, lower extremity swelling and skin ulcers were cure and the level of D-direct, eosinophil and platelet returned to normal. Pulmonary CTA and vascular color Doppler also suggested that thrombus was completely absorbed. Conclusions When HES in children complicated with D-dimer increasing, we need to investigate the possibility of deep vein thrombosis and organ embolism event; and once thrombosis was found, the application of anticoagulation and glucocorticoid therapy may be effective and can reduce the occurrence of severe thrombotic events.

关 键 词：嗜酸粒细胞增多综合征 儿童 肺栓塞 血小板减少 下肢深静脉血栓形成 

分 类 号：R725.5[医药卫生—儿科]