获得性血友病1例

P. Barlamov, E. R. Vasilyeva, M. E. Golubeva, V. G. Zhelobov, A. A. Shutylev, T. Kravtsova
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引用次数: 0

摘要

本文介绍获得性血友病a型获得性凝血因子VIII缺乏症的形成、诊断和治疗的临床病例。患者R, 71岁,2018年4月起,发现血原子型大血肿急性出血综合征,表现为下颌下区,颈部,胸部,右乳房,右侧耻骨和腹股沟区,左侧股骨前内侧面,左侧胫骨前表面。标准实验室检查,颈部、肺、腹部软组织的计算机断层扫描;coagulogram;凝血因子;在患者住院期间评估因子VIII抑制剂的动态;血小板聚集功能。典型的血友病型出血,凝血指标延长,存在抑制因子VIII (7,0 BAA),因子VIII降低(2%),可诊断为获得性血友病a。抗抑制凝血复合物,新鲜冷冻血浆成功用于治疗。患者正在地区血液学中心接受观察。血肿未复发。通过对A型获得性血友病的临床观察,探讨了A型获得性血友病的病程特点、诊断及治疗方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Clinical case of an acquired hemophilia
The aimof the work is to describe the clinical case of formation, diagnosis and treatment of the acquired form of blood clotting factor VIII deficiency – of acquired hemophilia A.Material and methods. Patient R., 71 years, from April 2018, was found an acute hemorrhagic syndrome in the hematomic type of large hematomas manifested submandibular region, neck, chest, right breast, pubic and inguinal regions on the right, the anterior-medial surface of the left femur, anterior surface of left tibia. Standard laboratory tests, computed tomography of soft tissues of the neck, lungs, abdomen; coagulogram; blood clotting factors; inhibitor of factor VIII were evaluated in dynamics during the patient’s stay in the hospital; platelet aggregation function.Results. Typical gematomny type of bleeding, prolongation of coagulation indicators, the presence of the inhibitor factor VIII (7,0 BAA), the decrease in factor VIII (2 %) allowed diagnosis of acquired hemophilia A. Anti-inhibitory coagulant complex, fresh frozen plasma was successfully used for treatment. The patient is under observation in the regional Hematology center. The hematomas were not renewed.Conclusion. Our clinical observation demonstrates the features of the course, the algorithm of diagnosis and management of patients with of acquired hemophilia A.
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