CAJ | 학술논문

目的总结国内单中心血管性血友病(VWD)的临床特征和诊治现状.方法回顾性分析162例VWD患者的临床资料和实验室检查结果.结果 162例患者中男76例[中位年龄7.2(2.0～41.0)岁]、女86例[中位年龄20.7(5.0～48.0)岁].1型VWD患者86例(53.1％),2型42例(25.9％,其中2A型33例、2M型4例、2N型5例),3型34例(21.0％).87例(53.7％)患者有明确出血性疾病家族史.出血表现以皮肤瘀斑(61.7％)、鼻出血(53.7％)、手术或外伤后出血不止(53.1％)为主,月经量多为女性患者常见主诉(66.3％).56例(34.6％)患者Vicenza出血评分为异常出血.45例(27.8％)患者接受血制品替代治疗,8例患者接受1-去氨基-8-右旋-精氨酸血管加压素治疗.57例月经量多的女性患者中,8例联合孕激素控制出血,2例行子宫切除术.结论 VWD临床表现异质性较高,应分步进行确诊及分型检查并实施个体化治疗.
목적 총결국내단중심혈관성혈우병(VWD)적림상특정화진치현상.방법 회고성분석162례VWD환자적림상자료화실험실검사결과.결과 162례환자중남76례[중위년령7.2(2.0～41.0)세]、녀86례[중위년령20.7(5.0～48.0)세].1형VWD환자86례(53.1％),2형42례(25.9％,기중2A형33례、2M형4례、2N형5례),3형34례(21.0％).87례(53.7％)환자유명학출혈성질병가족사.출혈표현이피부어반(61.7％)、비출혈(53.7％)、수술혹외상후출혈불지(53.1％)위주,월경량다위녀성환자상견주소(66.3％).56례(34.6％)환자Vicenza출혈평분위이상출혈.45례(27.8％)환자접수혈제품체대치료,8례환자접수1-거안기-8-우선-정안산혈관가압소치료.57례월경량다적녀성환자중,8례연합잉격소공제출혈,2례행자궁절제술.결론 VWD림상표현이질성교고,응분보진행학진급분형검사병실시개체화치료.
Objective To analyse the epidemiological feature,clinical characters and therapeutic regimens for von Willebrand disease (VWD).Methods The clinical data and laboratory tests results of 162 VWD patients in our center were analyzed.Results There were 76 males and 86 female among these patients with the mean age of 7.2(2.0-41.0) and 20.7(5.0-48.0) years,respectively.86 patients (53.1％) were identified to be type 1 VWD,34 patients (21.0％) type 3 VWD and 42 patients (25.9％) type 2 VWD.Among type 2 VWD patients,33 patients were type 2A,4 patients type 2M,5 patients type 2B.Eighty-seven patients (53.7％) had a definite family history of bleeding tendency.The most common and specific bleeding symptoms were easy bruising (61.7％),epistaxis (53.7％),prolonged bleeding after surgery or minor injury (53.1％).Menorrhagia (66.3％) was common in female patients.The analysis of Vicenza bleeding scores in all patients showed that only 56 (34.6％) patients had abnormal bleeding scores.FVIII/VWF concentrates and cryoprecipitate were applied to 45 patients (27.8％),Desmopressin (DDAVP) to 8 patients.Eight female patients need oral contraceptives jointly to control menorrhagia.Hysterectomy had to be performed in 2 female patients with VWD.Conclusion VWD was a common congenital bleeding disorder with heterogeneous characters,it was necessary to screen,identify,classify accurately this disease in order to supply to effectively individulized treatment.