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.
Zhongguo Dang Dai Er Ke Za Zhi. 2022 Jul 15;24(7):817-820. doi: 10.7499/j.issn.1008-8830.2203060.
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 K. 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.
一名2岁5个月大的男孩反复鼻出血，凝血功能检查显示活化部分凝血活酶时间（APTT）显著延长。进一步实验室检查显示，在APTT纠正试验中延长的APTT未立即得到纠正，狼疮抗凝物阳性且凝血酶原活性降低。该男孩被诊断为低凝血酶原血症-狼疮抗凝物综合征。经糖皮质激素、免疫球蛋白和维生素K治疗后病情改善。该男孩已随访6个月，未观察到鼻出血。凝血酶原活性恢复正常，狼疮抗凝物仍为阳性。这是一种相对罕见的疾病，对于有出血症状和凝血障碍的患者，建议进行APTT纠正试验、狼疮抗凝物检测和凝血因子稀释试验等检查，可提高该病的检出率，从而实现早期诊断，在早期提供合理治疗并改善预后。
