世界血友病同盟《全球血友病年度报告2022》解读  

Interpretation of the “2022 World Hemophilia Annual Report” by the World Federation of Hemophilia

作　　者：朱礼君 郑昌成 ZHU Lijun;ZHENG Changcheng(Department of Hematology,The First Affiliated Hospital of USTC Anhui Provincial Hospital,Anhui Hefei 230001,China)

机构地区：[1]中国科学技术大学附属第一医院(安徽省立医院)血液科,安徽合肥230001

出　　处：《诊断学理论与实践》2024年第1期40-45,共6页Journal of Diagnostics Concepts & Practice

摘　　要：世界血友病同盟(World Federation of Hemophilia,WFH)于2022年10月发布了新的年度血友病全球报告(Annual Global Survey,AGS)(以下简称AGS 2022),提出了新的工作目标。在2025年前,提高识别并诊断遗传性出血性疾病能力,包括:在原有基础性上,提高25%的血友病确诊率和14%的血管性血友病(von Willebrand disease,vWD)患者确诊率;提供合适的护理和治疗,特别是要提高25%的18岁以下重型血友病患者预防治疗率,继续每年至少为20000例患者提供WFH人道主义援助。AGS 2022预估了全球的血友病年平均患病率:血友病A(hemophilia A,HA)为17.1/10万(男性),其中重型HA为6.0/10万(男性);血友病B(hemophilia B,HB)为3.8/10万(男性),其中重型HB为1.1/10万男性。根据世界人口(79亿,40亿男性),预计全球血友病患病率在10.6/10万左右,预计全球有血友病患者830895例,其中约282266例为重型患者。我国的血友病患病率在(2.73~3.09)/10万,低于全球水平。同时,AGS 2022提示,全球女性血友病患者有11700例,女性血友病占血友病患者总人数的5%;女性vWD患者有54066例,占vWD总人数的56%,女性其他出血性疾病有34370例,提示vWD是女性最常见的出血性疾病。我国的HA患者共27689例,其中0~4岁占4%,5~13岁占21%,14~18岁占12%,19~44岁占43%,45岁以上占16%,年龄不确定者占4%。相对于中低收入国家,高收入国家对于轻型血友病的确诊率高(男性40%比12%;女性86%比23%),不确定性患者比例少,而低收入国家的血友病患者早逝风险很大。经济发达地区的艾美赛珠单抗预防治疗使用显著高于我国。我国相较于欧美国家在血友病及其他出血性疾病患者的诊断及治疗方面还有较大差距,临床医务工作者需提高对血友病及其他出血性疾病的认识并不断提高诊断和治疗能力。The World Federation of Hemophilia(WFH) released its new Annual Global Survey(AGS) in October 2022. The report sets goals for diagnosis and treatment of hereditary bleeding disorders, including a 25% increase in the diagnosis rate of hemophilia and a 14% increase in the diagnosis rate of von Willebrand disease(vWD) patient by 2025. It also aims to provide appropriate care and treatment particularly by increasing the rate of prophylactic treatment for severe hemophilia patients under the age of 18 by 25%, and continuing to provide humanitarian aid to at least 20 000 patients annually through the WFH. The AGS 2022 estimates the annual average prevalence of hemophilia worldwide, with hemophilia A(HA) at 17.1 per 100 000 males, including severe HA at 6.0 per 100 000 males;and hemophilia B(HB) at 3.8 per 100000 males, including severe HB at 1.1 per 100 000 males. Based on the world population(7.9 billion, with 4 billion males),it is estimated that the global prevalence of hemophilia is around 10.6 per 100 000, with a total of 830 895 individuals with hemophilia worldwide, of which approximately 282 266 are severe cases. The prevalence rate of hemophilia in China is between 2.73 and 3.09 per 100 000, which is lower than that of the global average. It is indicated in the report that there are 11 700 female hemophilia patients, accounting for 5% of the total number of hemophilia patients;and there are 54 066 female vWD patients, accounting for 56% of the total vWD population, and 34 370 cases of other bleeding disorders in females, highlighting that vWD is the most common bleeding disorder among women. In China, there are a total of 27 689 patients with HA, with the age distribution as follows: 4% aged 0-4 years, 21% aged 5-13 years, 12% aged 14-18 years,43% aged 19-44 years, 16% aged 45 and above, and 4% with an uncertain age. Compared with low-income countries, highincome countries have a higher diagnosis rate for mild hemophilia and fewer patients with uncertain diagnosis. Patients with hemophilia in low-income countr

关 键 词：世界血友病同盟 出血性疾病 血友病A 血友病B 

分 类 号：R554.1[医药卫生—血液循环系统疾病] R446.1[医药卫生—内科学]