Volume 20 Issue 10
Oct.  2022
Turn off MathJax
Article Contents
Article Navigation >  Chinese Journal of General Practice  > 2022  >  20(10): 1678-1681
GAO Fei, TIAN Miao, LI Guang, DU Ming-zhu, ZHOU Zhi-hui, SONG Yan-ping, DONG Chang-hu. A retrospective clinical study of 9 cases with autoimmune diseases combined acquired hemophilia A[J]. Chinese Journal of General Practice, 2022, 20(10): 1678-1681. doi: 10.16766/j.cnki.issn.1674-4152.002677
Citation: GAO Fei, TIAN Miao, LI Guang, DU Ming-zhu, ZHOU Zhi-hui, SONG Yan-ping, DONG Chang-hu. A retrospective clinical study of 9 cases with autoimmune diseases combined acquired hemophilia A[J]. Chinese Journal of General Practice, 2022, 20(10): 1678-1681. doi: 10.16766/j.cnki.issn.1674-4152.002677
shu

A retrospective clinical study of 9 cases with autoimmune diseases combined acquired hemophilia A

doi: 10.16766/j.cnki.issn.1674-4152.002677
  • 1.

    The Second Clinical Medical College, Shaanxi University of Chinese Medicine, Xianyang, Shaanxi 712046, China

Funds:

 2019JM-607

 20JK0604

  • Received Date: 2022-02-10
    Available Online: 2022-11-30
    Abstract
  •   Objective  To analyze and summarize the clinical features, laboratory examination results, treatment plan and curative effects of patients with autoimmune diseases combined with acquired hemophilia A, so as to improve the understanding of the disease.  Methods  Nine patients with autoimmune diseases combined acquired hemophilia A who were admitted by Xi ' an Central Hospital from January 2012 to December 2020 were selected, and their clinical features, laboratory examination results, treatment plans and curative effects were retrospectively analyzed.  Results  Among the 9 patients, 4 were males and 5 were females. The median age was 60.0 (42.0~72.5) years. Bleeding symptoms were heterogeneous, mainly skin bleeding, but also bleeding from important organs such as gastrointestinal bleeding and corpus luteum hemorrhage. In the treatment of elimination of inhibitors, glucocorticoid monotherapy was used in 4 cases (44.5%), cyclophosphamide and prednisone (CP) regimen in 1 case (11.1%), rituximab combined with CP regimen or cyclophosphamide, vincristine and prednisone (COP) regimen in 3 cases (33.3%), and cyclophosphamide monotherapy in 1 case (11.1%). Among them, 3 cases were complete remission (33.3%), 4 cases were partial remission (44.5%), and 2 cases (22.2%) were not remission, and the total effective rate was 77.8%.  Conclusion  Autoimmune diseases combined acquired hemophilia A have a rapid onset, heavy bleeding and heterogeneity. The use of immunosuppressive therapy is more effective. In some cases, the combination of rituximab on the basis of immunosuppressive therapy can also achieve better results. After immunosuppression and coagulation factor supplementation, the disease can be quickly controlled and the effect is good, but 44.5% of patients only achieved partial remission after one course of treatment, suggesting that to completely remove the inhibitors, systemic treatment is required for the primary disease, and multiple courses of treatment or longer-term immunosuppressive treatment are required.

     

    • FullText(HTML)
  • loading
    • References(20)
  • [1]
    PAI M. Acquired hemophilia A[J]. Hematol Oncol Clin North Am, 2021, 35(6): 1131-1142. doi: 10.1016/j.hoc.2021.07.007
    [2]
    WINDYGA J, BARAN B, ODNOCZKO E, et al. Treatment guidelines for acquired hemophilia A[J]. Ginekol Pol, 2019, 90(6): 353-364. doi: 10.5603/GP.2019.0063
    [3]
    HUNT S, ROBERTSON J, CONN J, et al. A low-dose rituximab regimen for first-line treatment of acquired haemophilia A[J]. Eur J Haematol, 2022, 108(1): 28-33. doi: 10.1111/ejh.13708
    [4]
    MINGOT-CASTELLANO E, PARDOS-GEA J, HAYA S, et al. Management of acquired hemophilia A: Results from the Spanish registry[J]. Blood Adv, 2021, 5(19): 3821-3829. doi: 10.1182/bloodadvances.2021004626
    [5]
    TIEDE A, COLLINS P, KNOEBL P, et al. International recommendations on the diagnosis and treatment of acquired hemophilia A[J]. Haematologica, 2020, 105(7): 1791-1801. doi: 10.3324/haematol.2019.230771
    [6]
    FRANCHINI M, SCHIAVULLI M, LIUMBRUNO G M. Hemostatic therapy as a management strategy for acquired hemophilia: What does the future hold?[J]. Expert Rev Hematol, 2021, 14(3): 263-270. doi: 10.1080/17474086.2021.1892483
    [7]
    GARCIA-CHAVEZ J, MAJLUF-CRUZ A. Acquired hemophilia[J]. Gac Med Mex, 2020, 156(1): 67-77.
    [8]
    TAKAHASHI W, ICHIKAWA M, FURUICHI S, et al. Acquired hemophilia A requiring plasma exchange and mechanical ventilation[J]. Rinsho Ketsueki, 2019, 60(3): 191-196.
    [9]
    兰秋丽, 李慧, 宁俞智, 等. 典型围产期获得性血友病甲1例报告及文献复习[J]. 中华全科医学, 2020, 18(9): 1609-1611. doi: 10.16766/j.cnki.issn.1674-4152.001573

    LAN Q L, LI H, NING Y Z, et al. A case report and literature review of typical perinatal acquired hemophilia A[J]. Chinese Journal of General Practice, 2020, 18(9): 1609-1611. doi: 10.16766/j.cnki.issn.1674-4152.001573
    [10]
    KNOEBL P, MARCO P, BAUDO F, et al. Demographic and clinical data in acquired hemophilia A: Results from the European Acquired Haemophilia Registry (EACH2)[J]. J Thromb Haemost, 2012, 10(4): 622-631. doi: 10.1111/j.1538-7836.2012.04654.x
    [11]
    TIEDE A, ZIEGER B, LISMAN T. Acquired bleeding disorders[J]. Haemophilia, 2021, 27(Suppl 3): 5-13.
    [12]
    FLAUMENHAFT E J, TERRY K, SUSANNE M. Retention of coagulation factors and storage of freeze-dried plasma[J]. Mil Med, 2021, 186(Suppl 1): 400-407.
    [13]
    VILLA D, SEHN L H, SAVAGE K J, et al. Bendamustine and rituximab as induction therapy in both transplant-eligible and-ineligible patients with mantle cell lymphoma[J]. Blood Advances, 2020, 4(15): 3486-3494. doi: 10.1182/bloodadvances.2020002068
    [14]
    SEHN L H, SALLES G. Diffuse large B-cell lymphoma[J]. N Engl J Med, 2021, 384(9): 842-858. doi: 10.1056/NEJMra2027612
    [15]
    ONISAI M, VLADAREANU A-M, SPINU A, et al. Idiopathic thrombocytopenic purpura (ITP)-new era for an old disease[J]. Rom J Intern Med, 2019, 57(4): 273-283.
    [16]
    BARCELLINI W, FATTIZZO B. How I treat warm autoimmune hemolytic anemia[J]. Blood, 2021, 137(10): 1283-1294. doi: 10.1182/blood.2019003808
    [17]
    KNOEBL P, THALER J, JILMA P, et al. Emicizumab for the treatment of acquired hemophilia A[J]. Blood, 2021, 137(3): 410-419. doi: 10.1182/blood.2020006315
    [18]
    DARTEVEL A, AUBANEL S, PICA G, et al. Acquired hemophilia A associated with chronic myelomonocytic leukemia successfully treated by rituximab and azacitidine[J]. La Revue de Medecine Interne, 2018, 39(8): 654-657. doi: 10.1016/j.revmed.2018.03.373
    [19]
    SCHEP S J, VAN DIJK W E M, BECKERS E A M, et al. Treatment of acquired hemophilia A, a balancing act: Results from a 27-year Dutch cohort study[J]. Am J Hematol, 2021, 96(1): 51-59.
    [20]
    COLLINS P, BAUDO F, KNOEBL P, et al. Immunosuppression for acquired hemophilia A: Results from the European Acquired Haemophilia Registry (EACH2)[J]. Blood, 2012, 120(1): 47-55. doi: 10.1182/blood-2012-02-409185
    • Relative Articles
    • Supplements(0)
    • Cited By
  • 加载中

Catalog

    通讯作者: 陈斌, bchen63@163.com
    • 1. 

      沈阳化工大学材料科学与工程学院 沈阳 110142

    1. 本站搜索
    2. 百度学术搜索
    3. 万方数据库搜索
    4. CNKI搜索

    Tables(2)

    Get Citation
    PDF
    XML

    Article Metrics

    Article views (243) PDF downloads(9) Cited by()
    Proportional views
    Related

    Address：No. 287, Changhuai Road, Bengbu, Anhui Province, 233004, P.R.ChinaphoneNo：0552-3066635； 0552-3051890Email：zhqkyx@163.com

    Copyright © Chinese Journal of General Practice皖ICP备2020018345号-2

    Supported by: Beijing Renhe Information Technology Co., Ltd.    

    /

    DownLoad:  Full-Size Img  PowerPoint
    Return
    Return