血栓性血小板减少性紫癜发病机制及诊疗进展

赵丽东; 王梅芳; 陈玙; 常姝婷; 呼小茹; 程芳芳; 杨林花

doi:10.13201/j.issn.1004-2806.2018.11.020

血栓性血小板减少性紫癜发病机制及诊疗进展

- 山西医科大学第二医院血液科(太原, 030001)
基金项目:
山西省科技创新团队(No:201605D131044-05)

山西医科大学大学生创新创业校级项目(No:20160256)

详细信息

通讯作者: 杨林花,E-mail:Yanglh5282@163.com

中图分类号: R558.2

收稿日期: 2018-01-10

Update on the diagnosis and treatment of thrombotic thrombocytopenic purpura

More Information

Corresponding author: , E-mail: Yanglh5282@163.com

Received Date: 10 January 2018

摘要

- 血栓性血小板减少性紫瘢 /
- 发病机制 /
- 诊断 /
- 治疗
Abstract: Thrombotic thrombocytopenic purpura（TTP）is a disease characterized by thrombocytopenia and microvascular thrombosis.The disease is acute,the condition is dangerous,and the early death rate can reach 90%.This article intends to review the pathogenesis,diagnosis and treatment progress of TTP in order to improve the understanding and treatment of TTP among clinicians and improve the prognosis of patients with TTP.
- thrombotic thrombocytopenic purpura /
- pathogenesis /
- diagnosis /
- treatment

HTML全文

参考文献(29)

[1]	阮长耿,余自强.2012版血栓性血小板减少性紫癜诊断与治疗中国专家共识解读[J].临床血液学杂志,2013,26(3):145-146.
[2]	Sadler JE.What's new in the diagnosis and pathophysiology of thrombotic thrombocytopenic purpura[J].Hematology,2015,2015:631-636.
[3]	Joly BS,Coppo P,Veyradier A.Thrombotic thrombocytopenic purpura[J].Blood,2017,129:2836-2846.
[4]	田利伟,李小丰,金鑫,等.血栓性血小板减少性紫癜患者3例分析[J].中国实验诊断学,2016,20(7):1183-1185.
[5]	Crawley JT,de Groot R,Xiang Y,et al.Unravelling the scussile bond:how ADAMTS13 recognizes and cleaves von Willebrand factor[J].Blood,2011,118:3212-3221.
[6]	Banno F,Kokame K,Okuda T,et al.Complete deficiency in ADAMTS13 is prothrombotic,but it alone is not sufficient to cause thrombotic thrombocytopenic purpura[J].Blood,2006,107:3161-3166.
[7]	张军,顾健.血栓性血小板减少性紫癜发病机制及治疗进展[J].临床血液学杂志,2016,29(1):73-76.
[8]	Levy GG,Nichols WC,Lian EC,et al.Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura[J].Nature,2001,413:488-494.
[9]	Lotta LA,Garagiola I,Palla R,et al.ADAMTS13 mutations and polymorphisms in congenital thrombotic thrombocytopenic purpura[J].Hum Mutat,2010,31:11-19.
[10]	Schiviz A,Wuersch K,Piskernik C,et al.A new mouse model mimicking thrombotic thrombocytopenic purpura:correction of symptoms by recombinant human ADAMTS13[J].Blood,2012,119:6128-6135.
[11]	George JN,Lopez JA,Konkle BA.N-Acetylcysteine:an old drug,a new insight,a potentially effective treatment for thrombotic thrombocytopenic purpura[J].Transfusion,2014,54:1205-1207.
[12]	Sayani FA,Abrams CS.How I treat refractory thrombotic thrombocytopenic purpura[J].Blood,2015,125:3860-3867.
[13]	Chen J,Reheman A,Gushiken FC,et al.N-acetylcysteine reduces the size and activity of von Willebrand factor in human plasma and mice[J].J Clin Invest,2011,121:593-603.
[14]	Rottenstreich A,Hochberg-Klein S,Rund D,et al.The role of N-acetylcysteine in the treatment of thrombotic thrombocytopenic purpura[J].J Thromb Thrombolysis,2016,41:678-683.
[15]	Shortt J,Oh DH,Opat SS.ADAMTS13 antibody depletion by bortezomib in thrombotic thrombocytopenic purpura[J].N Engl J Med,2013,368:90-92.
[16]	Mazepa MA,Raval JS,Moll S,et al.Bortezomib induces clinical remission and reduction of ADAMTS13 inhibitory antibodies in relapsed refractory idiopathic thrombotic thrombocytopenic purpura[J].Br J Haematol,2014,164:900-902.
[17]	Patriquin CJ,Thomas MR,Dutt T,et al.Bortezomib in the treatment of refractory thrombotic thrombocytopenic purpura[J].Br J Haematol,2016,173:779-785.
[18]	Eskazan AE.Bortezomib therapy in patients with relapsed/refractory acquired thrombotic thrombocytopenic purpura[J].Ann Hematol,2016,95:1751-1756.
[19]	Crawley JT,Scully MA.Thrombotic thrombocytopenic purpura:basic pathophysiology and therapeutic strategies[J].Hematology (Am Soc Hematol Educ Program),2013,2013:292-299.
[20]	Knobl P,Jilma B,Gilbert JC,et al.Anti-von Willebrand factor aptamer ARC1779 for refractory thrombotic thrombocytopenic purpura[J].Transfusion,2009,49:2181-2185.
[21]	Callewaert F,Roodt J,Ulrichts H,et al.Evaluation of efficacy and safety of the anti-VWF Nanobody ALX-0681 in a preclinical baboon model of acquired thrombotic thrombocytopenic purpura[J].Blood,2012,120:3603-3610.
[22]	Feys HB,Roodt J,Vandeputte N,et al.Inhibition of von Willebrand factor-platelet glycoprotein Ib interaction prevents and reverses symptoms of acute acquired thrombotic thrombocytopenic purpura in baboons[J].Blood,2012,120:3611-3614.
[23]	Peyvandi F,Scully M,Kremer Hovinga JA,et al.Caplacizumab for acquired thrombotic thrombocytopenic purpura[J].N Engl J Med,2016,374:511-522.
[24]	Scully M,Cataland SR.Results of the randomized,double-blind,placebo-controlled,phase 3 hercules study of caplacizumab in patients with acquired thrombotic thrombocytopenic purpura[J].Blood,2017,130(Suppl 1):LBA-1.
[25]	Scully M,Hunt BJ,Benjamin S,et al.Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies[J].Br J Haematol,2012,158:323-325.
[26]	Kremer Hovinga JA,Vesely SK,Terrell DR,et al.Survival and relapse in patients with thrombotic thrombocytopenic purpura[J].Blood,2010,115:1500-1511.
[27]	Bresin E,Gastoldi S,Daina E,et al.Rituximab as pre-emptive treatment in patient with thrpmbotic thrombocytopenic purpura and evidence of anti-ADAMTS13 autoantibodies[J].Thromb Haemost,2009,101:233-238.
[28]	Hie M,Gay J,Galicier L,et al.Preemptive rituximab infusions after remission efficiently prevent relapses in acquired thrombotic thrombocytopenic purpura[J].Blood,2014,124:204-210.
[29]	吕翠翠,孙甜甜,鞠满凯,等.57例血栓性血小板减少性紫癜患者的临床分析[J].临床血液学杂志,2015,28(11):957-965.