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Poster Networking

Inherited Bleeding Disorders and Inhibitors

PB1208 - A multicenter cross-sectional study of hemophilia B patients in the central and western regions of China

Tuesday, June 25, 2024
13:45 – 14:45 ICT
Room: Exhibition Hall

    Co-Author(s)

  • WW

    Wen Wang

    M.D.
    Huazhong University of Science and Technology Tongji Medical College Tongji Hospital
    wuhan, Hubei, China (People's Republic)

  • LX

    Li Xu

    Professor
    The Second People's Hospital of Anhui Province
    Hefei, Anhui, China (People's Republic)

  • JW

    Jingsheng Wu

    Physician
    Department of Hematology, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, China
    Hefei, Anhui, China (People's Republic)

  • WL

    Weiyong Liu

    Professor
    The First Affiliated Hospital of University of Science and Technology of China
    Hefei, Anhui, China (People's Republic)

  • JJ

    Jiao Jin

    Professor
    The Affiliated Hospital of Guizhou Medical University
    Guiyang, Guizhou, China (People's Republic)

  • JH

    Jing Huang

    Professor
    The Affiliated Hospital of Guizhou Medical University
    Guiyang, Guizhou, China (People's Republic)

  • ZX

    Zhongjin Xu

    Professor
    Jiangxi Provincial Children’s Hospital
    Nanchang, Jiangxi, China (People's Republic)

  • YH

    Yali Huang

    M.D.
    Jiangxi Provincial Children’s Hospital
    Nanchang, Jiangxi, China (People's Republic)

  • BL

    Bai Li

    M.D.
    The First Affiliated Hospital of Zhengzhou University
    Zhengzhou, Henan, China (People's Republic)

  • YL

    Yufeng Liu

    Professor
    The First Affiliated Hospital of Zhengzhou University
    Zhengzhou, Henan, China (People's Republic)

  • QZ

    Qing Zhang

    Professor
    Chengdu Women’s and Children’s Central Hospital, School of Medicine, University of Electronic Science and Technology of China
    Chengdu, Sichuan, China (People's Republic)

  • MZ

    Min Zhou

    Professor
    Chengdu Women’s and Children’s Central Hospital, School of Medicine, University of Electronic Science and Technology of China
    Chengdu, Sichuan, China (People's Republic)

  • JP

    Jie Peng

    Professor
    Xiangya Hospital Central South University
    Changsha, Hunan, China (People's Republic)

    • Presenting Author(s)

  • Qun Hu, MD

    professor
    Huazhong University of Science and Technology Tongji Medical College Tongji Hospital
    wuhan, Hubei, China (People's Republic)

Background: Patients with hemophilia experience spontaneous and trauma-induced bleeding, and recurrent joint bleeding can lead to ongoing functional loss and affect the patient's quality of life (QoL).

Aims: To study the current status of HB patients in the central and western regions of China.

Methods: This cross-sectional, multicenter study was conducted in seven provinces in the central and western regions of China from April 2019 to June 2023. Samples were collected for the activity of FⅨ, inhibitor, and gene mutation. Furthermore, six index joints’ status and quality of life (QoL) were assessed.

Results: A total of 185 HB patients with a mean age of 16.40 years were enrolled. 34.6% of HB patients had varying degrees of diagnostic delay while 37.3% experienced varying degrees of treatment delay. The incidence of inhibitors was 5.9%. We identified 123 genetic variants, with missense mutations being the most common (53.2%). 84.0% of HB mothers were genetically identified as carriers, with a median clotting factor level was 0.54 IU/mL. 70.8% of HB patients had a history of joint hemorrhage, with a target joint rate of 64.1%. Lower extremity joints were the most aberrant in patients. The Hemophilia Joint Health Score (HJHS) score was significantly positively correlated with the Hemophilia Early Arthropathy Detection with Ultrasound in China (HEAD-US-C) (r = 0.823, P < 0.001). Patients who received prevention treatment, inhibitor negative, without treatment delay, and high-intensity replacement therapy showed a higher total score on the short form-36 health survey (SF-36).

Conclusion(s): One-third of the HB patients had varying degrees of delay in diagnosis and treatment, and the incidence of inhibitors was 5.9%. HEAD-US-C and HJHS can complement each other in the evaluation to give a valid basis for early clinical management. Early detection and preventive treatment, along with reducing high-intensity replacement therapy and inhibitor generation, can effectively improve the QoL of patients.