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Management of refractory thrombotic thrombocytopenic purpura: a case report

Published on Jun. 25, 2023Total Views: 3394 timesTotal Downloads: 1500 timesDownloadMobile

Author: Yang ZHANG 1, 2 Tian-Feng HUA 1, 2 Yao ZHENG 1, 2 Wen-Yan XIAO 1, 2 Min YANG 1, 2

Affiliation: 1. The Second Department of Intensive Care Unit, the Second Affiliated Hospital of Anhui Medical University, Hefei 230601, China 2. The Laboratory of Cardiopulmonary Resuscitation and Critical Care Medicine, the Second Affiliated Hospital of Anhui Medical University, Hefei 230601, China

Keywords: Thrombotic thrombocytopenic purpura Rituximab Plasma exchange

DOI: 10.12173/j.issn.1004-5511.202206009

Reference: Zhang Y, Hua TF, Zheng Y, Xiao WY, Yang M. Management of refractory thrombotic thrombocytopenic purpura: a case report[J]. Yixue Xinzhi Zazhi, 2023, 33(3): 237-242. DOI: 10.12173/j.issn.1004-5511.202206009.[Article in Chinese]

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Abstract

Thrombotic thrombocytopenic purpura (TTP) is a rare disease causing a critical condition often involving multisystems and having a high mortality rate. The mainstays of treatment being plasma exchange (PEX) and corticosteroids . Refractory TTP is defined as a failure of platelet response after 5 PEX and corticosteroids therapy. This article reports a patient with refractory TTP who did not improve after a PEX and corticosteroids therapy, but was later successfully treated with rituximab.

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References

1.Joly BS, Coppo P, Veyradier A. Thrombotic thrombocytopenic purpura[J]. Blood, 2017, 129(21): 2836-2846. DOI: 10.1182/blood-2016-10-709857.

2.Tsai HM. Thrombotic thrombocytopenic purpura: beyond empiricism and plasma exchange[J]. Am J Med, 2019, 132(9): 1032-1037. DOI: 10.1016/j.amjmed.2019.03.009.

3.Zheng XL, Vesely SK, Cataland SR, et al. ISTH guidelines for treatment of thrombotic thrombocytope-nic purpura[J]. J Thromb Haemost, 2020, 18(10): 2496-2502. DOI: 10.1111/jth.15010.

4.中华医学会血液学分会血栓与止血学组. 血栓性血小板减少性紫癜诊断与治疗中国指南(2022年版)[J]. 中华血液学杂志, 2022, 43(1): 7-12. [Thrombosis and Hemostasis Group, Chinese Society of Hematology, Chinese Medical Association. Chinese guideline on the diagnosis and management of thrombotic thrombocy-topenic purpura (2022)[J]. Chinese Journal of Hematology, 2022, 43(1): 7-12.] DOI: 10.3760/cma.j.issn.0253-2727.2022.01.002.

5.Scully M, Cataland S, Coppo P, et al. Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies[J].  J Thromb Haemost, 2017, 15(2): 312-322. DOI: 10.1111/jth.13571.

6.Owattanapanich W, Wongprasert C, Rotchanapanya W, et al. Comparison of the long-term remission of rituximab and conventional treatment for acquired thrombotic thrombocytopenic purpura: a sys-tematic review and meta-analysis[J]. Clin Appl Thromb Hemost, 2019, 25: 1076029618825309. DOI: 10.1177/1076029618825309.

7.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.

8.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.

9.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.

10.Gutterman LA, Kloster B, Tsai HM. Rituximab therapy for refractory thrombotic thrombocytopenic purpura[J]. Blood Cells Mol Dis, 2002, 28(3): 385-391. DOI: 10.1006/bcmd.2002.0522.

11.Schieppati F, Russo L, Marchetti M, et al. Low levels of ADAMTS-13 with high anti-ADAMTS-13 anti-bodies during remission of immune-mediated thrombotic thrombocytopenic purpura highly predict for disease relapse: a multi-institutional study[J]. Am J Hematol, 2020, 95(8): 953-959. DOI: 10.1002/ajh.25845.

12.张炎,张路,陈苗,等.含利妥昔单抗联合方案治疗难治/复发血栓性血小板减少性紫癜10例临床分析[J].中华血液学杂志, 2018, 39(10): 855-858. [Zhang Y, Zhang L, Chen M, et al. Efficacy and safety of rituximab-contained regimen for refractory and relapsing thrombotic thrombocytopenic purpura: a retrospective study of 10 cases[J]. Chinese Journal of Hematology, 2018, 39(10): 855-858.] DOI: 10.3760/cma.j.issn.0253-2727. 2018.10.013.

13.Page EE, Kremer Hovinga JA, Terrell DR, et al. Rituximab reduces risk for relapse in patients with thrombotic thrombocytopenic purpura[J]. Blood, 2016, 127(24): 3092-3094. DOI: 10.1182/blood-2016-03-703827.

14.王静,吴天勤,沈红石,等.利妥昔单抗治疗特发性血栓性血小板减少性紫癜的临床研究[J].中华血液学杂志, 2015, 36(4): 316-320. [Wang J, Wu TQ, Shen HS, et al. Clinical study on Rituximab in the treatment of idiopathic thrombotic thrombocytopenic purpura[J]. Chinese Journal of Hematology, 2015, 36(4): 316-320.] DOI: 10.3760/cma.j.issn.0253-2727.2015.04.012.

15.Zwicker JI, Muia J, Dolatshahi L, et al. Adjuvant low-dose rituximab and plasma exchange for acquired TTP[J]. Blood, 2019, 134(13): 1106-1109. DOI: 10.1182/blood. 2019000795.

16.Scully M, Cataland SR, Peyvandi F, et al. Caplacizumab treatment for acquired thrombotic thrombocy-topenic purpura[J]. N Engl J Med, 2019, 380(4): 335-346. DOI: 10.1056/NEJMoa1806311.

17.Khan S, Landry K, Umyarova E. Caplacizumab treatment for acquired refractory thrombotic thrombo-cytopenic purpura[J]. Br J Haematol, 2020, 191(2): e44-e46. DOI: 10.1111/bjh.16977.

18.Mellaza C, Henry N, Fayolle PM, et al. Refractory auto-immune thrombotic thrombocytopenic pupura successfully treated with caplacizumab[J]. Front Med (Lausanne), 2020, 7: 549931. DOI: 10.3389/fmed.2020.549931.

19.Joly BS, Coppo P, Veyradier A. An update on pathogenesis and diagnosis of thrombotic thrombocy-topenic purpura[J].  Expert Rev Hematol, 2019, 12(6): 383-395. DOI: 10.1080/17474086.2019.1611423.

20.Graça NAG, Joly BS, Voorberg J, et al. TTP: From empiricism for an enigmatic disease to targeted molecular therapies[J]. Br J Haematol, 2022, 197(2): 156-170. DOI: 10.1111/bjh.18040.