摘要
Abstract
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 di-agnosis 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 an-nually 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 100 000 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 be-tween 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 fe-male vWD patients,accounting for 56%of the total vWD population,and 34 370 cases of other bleeding disorders in fe-males,highlighting that vWD is the most common bleeding disorder among women.In China,there are a total of 27 689 pa-tients 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,high-income countries have a higher diagnosis rate for mild hemophilia and fewer patients with uncertain diagnosis.Patients with hemophilia in low-income countries are at a significant risk of early death.The use of emicizumab for prophylactic treat-ment in economically developed regions is significantly higher than that in China.There is still a considerable gap between China and European and American countries in the diagnosis and treatment of hemophilia and other bleeding disorders.It is necessary for clinical professionals in China to enhance their understanding of hemophilia and other bleeding disorders and to continuously improve diagnosis and treatment capabilities.关键词
世界血友病同盟/出血性疾病/血友病A/血友病BKey words
World Federation of Hemophilia/Bleeding disease/Hemophilia A/Hemophilia B分类
医药卫生
