Volume 20 Issue 4
Apr.  2022
Turn off MathJax
Article Contents
Article Navigation >  Chinese Journal of General Practice  > 2022  >  20(4): 598-601
WANG Tian-tian, CAO Jun-jie, LIU Xu-hui, FAN Zheng, PEI Ren-zhi, LU Ying. Clinical characteristics and treatment of thrombotic thrombocytopenic purpura secondary to connective tissue disease[J]. Chinese Journal of General Practice, 2022, 20(4): 598-601. doi: 10.16766/j.cnki.issn.1674-4152.002409
Citation: WANG Tian-tian, CAO Jun-jie, LIU Xu-hui, FAN Zheng, PEI Ren-zhi, LU Ying. Clinical characteristics and treatment of thrombotic thrombocytopenic purpura secondary to connective tissue disease[J]. Chinese Journal of General Practice, 2022, 20(4): 598-601. doi: 10.16766/j.cnki.issn.1674-4152.002409
shu

Clinical characteristics and treatment of thrombotic thrombocytopenic purpura secondary to connective tissue disease

doi: 10.16766/j.cnki.issn.1674-4152.002409

  • Department of Hematology, the Affiliated People ' s Hospital of Ningbo University, Ningbo, Zhejiang 315040, China

Funds:

 2017KY144

  • Received Date: 2021-08-21
    Available Online: 2022-08-20
    Abstract
  •   Objective  To analyze the clinical characteristics and treatment of thrombotic thrombocytopenic purpura (TTP) secondary to connective tissue disease (CTD) and explored the treatments of TTP.  Methods  We retrospectively reviewed clinical manifestations, laboratory examinations and treatment, efficacy, and survival of 8 TTP patients secondary to CTD admitted to the Affiliated People ' s Hospital of Ningbo University from January 2014 to June 2017.  Results  All 8 patients with TTP were diagnosed with CTD for the first time. All patients presented with acute onset and atypical clinical presentation. ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 motif, member 13, ADAMTS13) activities were 0 and its inhibitors were positive. All the patients received the treatment of plasma exchange plus steroid (1-2 mg/kg). One patient ' s platelet count increased after treatment, and then gave up treatment. In rest 7 patients, the median time of platelet recovered to normal after treatment was 12 (4-18) days, ADAMTS13 activity returned to 100% and ADAMTS13 antibody turned negative in 1 month after the treatment. Seven cases received treatment with cyclosporine, azathioprine or cyclophosphamide later. Three patients received maintenance therapy with rituximab 100 mg every 3 months. Seven patients remained in hematologic remissions. Among them, the activity of ADAMTS13 decreased to 30% and its inhibitors were positive in 2 patients at 6 months after the treatment by routine monitoring. ADAMTS13 activity returned to normal adding a small dose of rituximab therapy after 1 month.  Conclusion  TTP secondary to CTD has an atypical clinical manifestations, high clinical suspicion or diagnosis of TTP, as soon as possible to improve ADAMTS13 activity and inhibitor determination, and small dose of rituximab combined with plasma exchange is given as early as possible. Low-dose rituximab maintenance therapy is given to reduce relapse.

     

    • FullText(HTML)
  • loading
    • References(22)
  • [1]
    BALASUBRAMANIYAM N, YANDRAPALLI S, KOLTE D, et al. Cardiovascular complications and their association with mortality in patients with thrombotic thrombocytopenic purpura[J]. Am J Med, 2021, 134(2): e89-e97. doi: 10.1016/j.amjmed.2020.06.020
    [2]
    包海燕, 乔渝森, 吴德沛. 获得性血栓性血小板减少性紫癜的研究进展[J]. 中华血液学杂志, 2017, 38(5): 440-443. https://www.cnki.com.cn/Article/CJFDTOTAL-XYSY201804052.htm

    BAO H Y, QIAO Y S, WU D P, Progress of acquired thrombotic thrombocytopenic purpura[J]. Chin J Hematol, 2017, 38(5): 440-443. https://www.cnki.com.cn/Article/CJFDTOTAL-XYSY201804052.htm
    [3]
    KANGRO K, ROOSE E, JOLY B, et al. Anti-ADAMTS13 autoantibody profiling in patients with immune-mediated thrombotic thrombocytopenic purpura[J]. Blood Adv, 2021, 5(17): 3427-3435. doi: 10.1182/bloodadvances.2020004172
    [4]
    ROOSE E, VEYRADIER A, VANHOORELBEKE K, et al. Insights into ADAMTS13 structure: Impact on thrombotic thrombocytopenic purpura diagnosis and management[J]. Curr Opin Hematol, 2020, 27(5): 320-326. doi: 10.1097/MOH.0000000000000602
    [5]
    PAGLIARI M, CAIRO A, BOSCARINO M, et al. Role of ADAMTS13, VWF and F8 genes in deep vein thrombosis[J]. PLoS One, 2021, 16(10): e0258675. DOI: 10.1371/journal.pone.0258675.
    [6]
    LI J, JIANG J, WANG C, et al. Clinical features and prognosis of patients with thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus: A review of 25 cases[J]. Ital J Pediatr, 2019, 45(1): 55-60. doi: 10.1186/s13052-019-0641-y
    [7]
    SHARMA P, GURUNG A, DAHAL S, et al. Connective Tissue disorders in patients with thrombotic thrombocytopenic purpura: A retrospective analysis using a national database[J]. J Clin Med Res, 2019, 11(7): 509-514. doi: 10.14740/jocmr3850
    [8]
    RORIZ M, LANDAIS M, DESPREZ J, et al. Risk factors for autoimmune diseases development after thrombotic thrombocytopenic purpura[J]. Medicine, 2015, 94(42): e1598. doi: 10.1097/MD.0000000000001598
    [9]
    BARNAS J, LOONEY R, ANOLIK J J, et al. B cell targeted therapies in autoimmune disease[J]. Adv Exp Med Biol, 2019, 12(61): 92-99.
    [10]
    BALDUINI C, GUGLIOTTA L, LUPPI M, et al. High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: A randomized study[J]. Ann Hematol, 2010, 89(6): 591-596. doi: 10.1007/s00277-009-0877-5
    [11]
    王荷花, 陈慧珍, 童秀珍, 等. 21例血栓性血小板减少性紫癜的临床分析[J]. 中华全科医学, 2010, 8(10): 1224-1226. https://www.cnki.com.cn/Article/CJFDTOTAL-SYQY201010011.htm

    WANG H H, CHEN H Z, TONG X Z, et al, Clinical Analysis of 21 Patents with of 21 Patients with Thromobotic Thrombocytopenic Purpura[J]. CHinese Journal of Genneral Practi, 2010, 8(10): 1224-1226. https://www.cnki.com.cn/Article/CJFDTOTAL-SYQY201010011.htm
    [12]
    LI X, MO X, HUANG G, et al. Therapeutical plasma exchange for thrombotic thrombocytopenic purpura in the emergency department: A single center experience[J]. Am J Emerg Med, 2020, 12(11): 19.
    [13]
    THUMMA S, IDRESS S, PHUYAL P, et al. When the standard treatment fails: Rituximab therapy for refractory TTP[J]. Am J Ther, 2019, 26(4): e552-e553. doi: 10.1097/MJT.0000000000000808
    [14]
    SUN L, MACK J, LI A, et al. Predictors of relapse and efficacy of rituximab in immune thrombotic thrombocytopenic purpura[J]. Blood Adv, 2019, 3(9): 1512-1518. doi: 10.1182/bloodadvances.2019031039
    [15]
    REDDY M, HOFMANN S, SHEN Y, et al. Comparison of low fixed dose versus standard-dose rituximab to treat thrombotic thrombocytopenic purpura in the acute phase and preemptively during remission[J]. Transfus Apher Sci, 2020, 59(6): 102885. DOI: 10.1016/j.transci.2020.102885.
    [16]
    FALTER T, HEROLD S, WEYER-ELBERICH V, et al. Relapse rate in survivors of acute autoimmune thrombotic thrombocytopenic purpura treated with or without rituximab[J]. Thromb Haemost, 2018, 118(10): 1743-1751. doi: 10.1055/s-0038-1668545
    [17]
    SCULLY M, MCDONALD V, CAVENAGH J, et al. A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura[J]. Blood, 2011, 118(7): 1746-1753. doi: 10.1182/blood-2011-03-341131
    [18]
    王夕妍, 刘晓帆, 薛峰, 等. 血栓性血小板减少性紫癜83例临床分析[J]. 中华血液学杂志, 2020, 41(3): 216-221. https://www.cnki.com.cn/Article/CJFDTOTAL-SANE202104028.htm

    WANG X Y, LIU X F, XUE F, et al, Clinical characteristics of 83 patients with thrombotic thrombocytopenic purpura[J], Chin J Hematol, 2020, 41(3): 216-221. https://www.cnki.com.cn/Article/CJFDTOTAL-SANE202104028.htm
    [19]
    KUBO M, SAKAI K, YOSHⅡ Y, et al. Rituximab prolongs the time to relapse in patients with immune thrombotic thrombocytopenic purpura: Analysis of off-label use in Japan[J]. Int J Hematol, 2020, 112(6): 764-772. doi: 10.1007/s12185-020-02974-y
    [20]
    JESTIN M, BENHAMOU Y, SCHELPE A, et al. Preemptive rituximab prevents long-term relapses in immune-mediated thrombotic thrombocytopenic purpura[J]. Blood, 2018, 132(20): 2143-2153. doi: 10.1182/blood-2018-04-840090
    [21]
    MA W, BAI W, WU X, et al. Successful treatment of refractory thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus with combination of plasma exchange and low-dose rituximab[J]. Lupus, 2020, 29(14): 1961-1967. doi: 10.1177/0961203320953473
    [22]
    贾茜, 何杨, 阮长耿. 获得性血栓性血小板减少性紫癜的研究进展[J]. 中国实验血液学杂志, 2018, 26(4): 1330-1234. https://www.cnki.com.cn/Article/CJFDTOTAL-XYSY201804052.htm

    JIA Q, HE Y, RUAN C G, Research advances of acquired thrombotic thrombocytopenic purpura[J]. Journal of Experimental Hematology, 2018, 26(4): 1330-1234. https://www.cnki.com.cn/Article/CJFDTOTAL-XYSY201804052.htm
    • 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(1)

    Get Citation
    PDF
    XML

    Article Metrics

    Article views (370) PDF downloads(8) 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