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Outcome of CARE: a 6-year national registry of acquired haemophilia A in China.

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收录情况： ◇ SCIE

作者：
Sun Boyang[1] * ; Xue Feng[1,*1] ; Feng Ying[2] ; Sun Jing[3] ; Yu Ziqiang[4] ; Hou Ming[5] ; Zhang Jingyu[6] ; Zeng Xiaojing[7] ; Zhao Yongqiang[8] ; Lian Shimei[9] ; Huang Meijuan[10] ; Niu Ting[11] ; Cui Zhongguang[12] ; Wu Jingsheng[13] ; Yang Renchi[1,*1] ;
机构： [1]State Key Laboratory of Experimental Haematology, National Clinical Research Centre for Haematological Disorders, Institute of Haematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Tianjin Laboratory of Blood Disease Gene Therapy, CAMS Key Laboratory of Gene Therapy for Blood Diseases, Tianjin, [2]Department of Haematology, Second Affiliated Hospital of Guangzhou Medical University, [3]Nanfang Hospital, Nanfang Medical University, Guangzhou, [4]Department of Haematology, The First Affiliated Hospital of Soochow University, Suzhou, [5]Department of Haematology, Qilu Hospital, Shandong University, Jinan, Shandong, [6]Department of Haematology, Key Laboratory of Haematology of Hebei Province, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei, [7]Department of Blood Transfusion, The Affiliated Hospital of Guizhou University, Guiyang, [8]Department of Haematology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, [9]Department of Haematology, Dalian Municipal Central Hospital, Affiliated Hospital of Dalian Medical University, Dalian, China, [10]Fujian Institute of Haematology, Fujian Medical University Union Hospital, Fuzhou, [11]Department of Haematology, West China Hospital, Sichuan University, Chengdu, Sichuan, [12]The Affiliated Hospital of Qingdao University, Qingdao and [13]Department of Haematology, Anhui Provincial Hospital, Anhui Medical University, Hefei, China
出处：
DOI： 10.1111/bjh.16128
ISSN：

关键词： acquired haemophilia A registry diagnosis haemostatic treatment immunosuppression outcome

摘要：
Acquired haemophilia A (AHA) is a rare haemorrhagic disorder caused by autoantibodies directed against the functional epitopes of coagulation factor VIII (FVIII). Its management relies on prompt diagnosis, control of bleeding and eradication of the inhibitor by immunosuppression. China Acquired Hemophilia Registry (CARE), a nationwide multicentre registry, was intended to survey the status of AHA and standardize its diagnosis and therapy in China. One hundred and eighty-seven registered patients had an average age of 52 years. Diagnosis was delayed in 46·5% patients. There was a significant delay from diagnosis to immunosuppressive therapy in 68·3% patients. Bleeding control was significantly higher in patients treated with prothrombin complex concentrate (PCC) versus FVIII replacement therapy (84·6% vs. 34·4%; P < 0·001). Inhibitor eradication with a combination of steroids and cyclophosphamide showed a higher partial remission (PR) rate (92·2% vs. 70·3%) and stable complete remission (CR) rate (82·8% vs. 48·6%) than with steroids alone. Logistic regression model showed age and malignancy were significantly related to survival at final follow-up. The mean age for the survivors [51 years (IQR, 35-65 years)] was significantly lower than that of the non-survivors [79 years (IQR, 67-86 years)] (P < 0·001). Overall survival was higher in non-malignancy group than malignancy group (94·9% vs. 70%) (OR = 1·313; 95% CI, 0·913-1·889, P = 0·015). © 2019 British Society for Haematology and John Wiley & Sons Ltd.

基金：
National Natural Science Foundation of China (81670118), Tianjin Municipal Science and Technology Commission Grant (15ZXLCSY00010, 18JCZDJ C35000, 16JCYBJC26700), CAMS Innovation Fund for Medical Sciences (CIFMS) (2016-I2M-1-002, 2017-I2M-3-018) and Novo Nordisk (China) Pharmaceuticals Co., Ltd. The authors appreciate the contribution of the participating centres (Appendix S1) of CARE to our study.
语种：
外文
被引次数：
WOS：
PubmedID：
中科院(CAS)分区：
出版当年[2019]版：
大类 | 2 区 医学
小类 | 2 区 血液学
最新[2023]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[2023]版：
Q1 HEMATOLOGY

影响因子： 最新[2023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：
第一作者机构： [1]State Key Laboratory of Experimental Haematology, National Clinical Research Centre for Haematological Disorders, Institute of Haematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Tianjin Laboratory of Blood Disease Gene Therapy, CAMS Key Laboratory of Gene Therapy for Blood Diseases, Tianjin,
通讯作者：
通讯机构： [1]State Key Laboratory of Experimental Haematology, National Clinical Research Centre for Haematological Disorders, Institute of Haematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Tianjin Laboratory of Blood Disease Gene Therapy, CAMS Key Laboratory of Gene Therapy for Blood Diseases, Tianjin, [*1]State Key Laboratory of Experimental Haematology, National Clinical Research Centre for Haematological disorders, Institute of Haematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Tianjin Laboratory of Blood Disease Gene Therapy, CAMS Key Laboratory of Gene Therapy for Blood Diseases, Tianjin, China
推荐引用方式(GB/T 7714)：
Sun Boyang,Xue Feng,Feng Ying,et al.Outcome of CARE: a 6-year national registry of acquired haemophilia A in China.[J].BRITISH JOURNAL OF HAEMATOLOGY.2019,187(5):653-665.doi:10.1111/bjh.16128.
APA：
Sun Boyang,Xue Feng,Feng Ying,Sun Jing,Yu Ziqiang...&Yang Renchi.(2019).Outcome of CARE: a 6-year national registry of acquired haemophilia A in China..BRITISH JOURNAL OF HAEMATOLOGY,187,(5)
MLA：
Sun Boyang,et al."Outcome of CARE: a 6-year national registry of acquired haemophilia A in China.".BRITISH JOURNAL OF HAEMATOLOGY 187..5(2019):653-665

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