2岁男孩反复鼻衄伴凝血功能异常  

Recurrent epistaxis with coagulation disorders in a boy aged 2 years

作　　者：李佳倬 田鑫[1] 廖楚舒 贺湘玲[1] 朱呈光[1] LI Jia-Zhuo;TIAN Xin;LIAO Chu-Shu;HE Xiang-Ling;ZHU Cheng-Guang(Department of Hematology and Oncology,First Affiliated Hospital of Hunan Normal University/Children's Medical Center,Hunan Provincial People's Hospital,Changsha 410005,China)

机构地区：[1]湖南师范大学附属第一医院/湖南省人民医院儿童医学中心血液肿瘤科,湖南长沙410005 [2]湖南师范大学附属第一医院/湖南省人民医院输血科,湖南长沙410005

出　　处：《中国当代儿科杂志》2022年第7期817-820,共4页Chinese Journal of Contemporary Pediatrics

摘　　要：2岁5个月男孩,反复鼻衄,凝血功能发现活化部分凝血活酶时间(activated partial thromboplastin time,APTT)明显延长,进一步实验室检查提示APTT纠正试验为即刻不能纠正,狼疮抗凝物检测阳性,凝血酶原活性低,诊断为低凝血酶原血症-狼疮抗凝物综合征。予激素、丙种球蛋白、维生素K1治疗后病情好转,随访至今已6个月,未再出现鼻衄,凝血酶原活性恢复正常,狼疮抗凝物检测仍为阳性。该病较罕见,临床上有出血症状伴凝血功能异常者,建议积极完善APTT纠正试验、狼疮抗凝物、凝血因子稀释试验等检查,可提高该病的检出率,从而早期诊断,早期合理治疗,更好地改善患儿预后。A boy,aged 2 years and 5 months,had recurrent epistaxis,and the coagulation function examination showed that activated partial thromboplastin time(APTT)was significantly prolonged.Further laboratory examinations showed that the prolonged APTT was not immediately corrected in the APTT correction test,with positive lupus anticoagulant and low prothrombin activity.The boy was diagnosed with hypoprothrombinemia-lupus anticoagulant syndrome.The condition was improved after treatment with glucocorticoid,immunoglobulin,and vitamin K1.The boy has been followed up for 6 months,and no epistaxis was observed.Prothrombin activity returned to normal,and lupus anticoagulant remained positive.This is a relatively rare disease,and for patients with bleeding symptoms and coagulation disorders,it is recommended to perform the tests such as APTT correction test,lupus anticoagulant testing,and coagulation factor dilution test,which can improve the detection rate of this disease,so as to achieve early diagnosis,provide rational treatment in the early stage,and improve the prognosis.

关 键 词：低凝血酶原血症-狼疮抗凝物综合征 凝血功能 鼻衄 儿童 

分 类 号：R725.5[医药卫生—儿科]