高级检索
当前位置: 首页 > 详情页

Patients with haemophilia A with inhibitors in China: a national real-world analysis and follow-up

文献详情

资源类型:
WOS体系:
Pubmed体系:

收录情况: ◇ SCIE

单位: [1]Chinese Acad Med Sci & Peking Union Med Coll, State Key Lab Expt Hematol, Natl Clin Res Ctr Blood Dis, Inst Hematol, 288 Nanjing Rd, Tianjin 300020, Peoples R China [2]Chinese Acad Med Sci & Peking Union Med Coll, Tianjin Lab Blood Dis Gene Therapy, CAMS Key Lab Gene Therapy Blood Dis,Blood Dis Hos, CAMS Ctr Cell Med,PUMC Dept Stem Cell & Regenerat, 288 Nanjing Rd, Tianjin 300020, Peoples R China [3]Univ Calgary, Cumming Sch Med, Dept Med, Calgary, AB, Canada [4]Univ Calgary, Cumming Sch Med, Dept Pediat, Calgary, AB, Canada [5]Univ Calgary, Cumming Sch Med, Dept Oncol, Calgary, AB, Canada [6]Alberta Hlth Serv, Southern Alberta Rare Blood & Bleeding Disorders, Foothills Med Ctr, Calgary, AB, Canada [7]Shandong Blood Ctr, Jinan, Peoples R China [8]Chinese Univ Sci & Technol, Affiliated Hosp 1, Hefei, Peoples R China [9]Guizhou Med Univ, Affiliated Hosp, Guiyang, Peoples R China [10]Capital Med Univ, Beijing Childrens Hosp, Beijing, Peoples R China [11]Huazhong Univ Sci & Technol,Tongji Hosp,Dept Pediat Hematol,Tongji Med Coll,Wuhan,Peoples R China [12]Kunming Med Univ, Affiliated Hosp 1, Kunming, Yunnan, Peoples R China [13]Xinjiang Uygur Autonomous Reg Peoples Hosp, Urumqi, Peoples R China
出处:
ISSN:

关键词: haemophilia A inhibitor haemostatic agents immune tolerance induction outcome China

摘要:
The development of alloantibodies (inhibitors) against coagulation factor VIII (FVIII) is the most serious complication of FVIII replacement therapy in patients with haemophilia A (HA). We carried out a nationwide study focussing on patients with HA with inhibitors in China to evaluate the condition and management of this population. The study retrospectively analysed patient characteristics, clinical history, manifestation, treatment strategy as well as individual haemophilia care of 493 patients with inhibitors (466 with severe HA and 27 with non-severe HA) registered all over China. The median (interquartile range) age at diagnosis of FVIII inhibitors was 13 (5-28) years in patients with severe HA and 24 (10 center dot 5-39 center dot 5) years in patients with non-severe HA. Most patients (85%) had high-titre inhibitors. Prothrombin complex concentrate and recombinant activated coagulation factor VII were used respectively in 76 center dot 2% and 29 center dot 2% of patients for acute bleeding. Only 22 center dot 3% of patients underwent immune tolerance induction (ITI) treatment, of whom 64 center dot 9% achieved negative inhibitor titre. In patients who did not undergo ITI, the inhibitors turned negative in 17 center dot 7%, and patients with low peak inhibitor titre were more likely to acquire negative titre spontaneously (odds ratio 11 center dot 524, 95% confidence interval 5 center dot 222-25 center dot 432; P = 0 center dot 000). We recorded that 3 center dot 2% of the patients died from haemophilia-related life-threatening bleeding.

基金:
语种:
被引次数:
WOS:
PubmedID:
中科院(CAS)分区:
出版当年[2020]版:
大类 | 2 区 医学
小类 | 2 区 血液学
最新[2025]版:
大类 | 2 区 医学
小类 | 2 区 血液学
JCR分区:
出版当年[2019]版:
Q1 HEMATOLOGY
最新[2024]版:
Q1 HEMATOLOGY

影响因子: 最新[2024版] 最新五年平均 出版当年[2019版] 出版当年五年平均 出版前一年[2018版] 出版后一年[2020版]

第一作者:
第一作者单位: [1]Chinese Acad Med Sci & Peking Union Med Coll, State Key Lab Expt Hematol, Natl Clin Res Ctr Blood Dis, Inst Hematol, 288 Nanjing Rd, Tianjin 300020, Peoples R China [2]Chinese Acad Med Sci & Peking Union Med Coll, Tianjin Lab Blood Dis Gene Therapy, CAMS Key Lab Gene Therapy Blood Dis,Blood Dis Hos, CAMS Ctr Cell Med,PUMC Dept Stem Cell & Regenerat, 288 Nanjing Rd, Tianjin 300020, Peoples R China
通讯作者:
通讯机构: [1]Chinese Acad Med Sci & Peking Union Med Coll, State Key Lab Expt Hematol, Natl Clin Res Ctr Blood Dis, Inst Hematol, 288 Nanjing Rd, Tianjin 300020, Peoples R China [2]Chinese Acad Med Sci & Peking Union Med Coll, Tianjin Lab Blood Dis Gene Therapy, CAMS Key Lab Gene Therapy Blood Dis,Blood Dis Hos, CAMS Ctr Cell Med,PUMC Dept Stem Cell & Regenerat, 288 Nanjing Rd, Tianjin 300020, Peoples R China
推荐引用方式(GB/T 7714):
APA:
MLA:

资源点击量:622 今日访问量:0 总访问量:452 更新日期:2025-07-01 建议使用谷歌、火狐浏览器 常见问题

版权所有:重庆聚合科技有限公司 渝ICP备12007440号-3 地址:重庆市两江新区泰山大道西段8号坤恩国际商务中心16层(401121)