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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Management of refractory thrombotic thrombocytopenic purpura: a case report
Published on Jun. 25, 2023Total Views: 3683 timesTotal Downloads: 1575 timesDownloadMobile
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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.
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