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ISTH guidelines for treatment of thrombotic thrombocytopenic purpura.国际血栓与止血学会（ISTH）血栓性血小板减少性紫癜治疗指南。

J Thromb Haemost. 2020 Oct;18(10):2496-2502. doi: 10.1111/jth.15010. Epub 2020 Sep 11.

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Thrombotic thrombocytopenic purpura: diagnostic criteria, clinical features, and long-term outcomes from 1995 through 2015.血栓性血小板减少性紫癜：1995年至2015年的诊断标准、临床特征及长期预后

Blood Adv. 2017 Apr 6;1(10):590-600. doi: 10.1182/bloodadvances.2017005124. eCollection 2017 Apr 11.

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Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study.用于快速评估血栓性微血管病成人患者的PLASMIC评分的推导与外部验证：一项队列研究

Lancet Haematol. 2017 Apr;4(4):e157-e164. doi: 10.1016/S2352-3026(17)30026-1. Epub 2017 Mar 2.

4

Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathy.伴有严重ADAMTS13缺乏的成人期血栓性微血管病（血栓性血小板减少性紫癜）的流行病学和病理生理学：法国全国血栓性微血管病登记处的横断面分析

Lancet Haematol. 2016 May;3(5):e237-45. doi: 10.1016/S2352-3026(16)30018-7. Epub 2016 Apr 16.

5

Cardiac troponin-I on diagnosis predicts early death and refractoriness in acquired thrombotic thrombocytopenic purpura. Experience of the French Thrombotic Microangiopathies Reference Center.心肌肌钙蛋白 I 有助于诊断，可预测获得性血栓性血小板减少性紫癜的早期死亡和难治性。法国血栓性微血管病参考中心的经验。

J Thromb Haemost. 2015 Feb;13(2):293-302. doi: 10.1111/jth.12790. Epub 2014 Dec 18.

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Rabies vaccine-associated thrombotic thrombocytopenic purpura.狂犬病疫苗相关血栓性血小板减少性紫癜

Transfus Med. 2014 Dec;24(6):428-9. doi: 10.1111/tme.12160. Epub 2014 Nov 10.

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Acute thrombotic thrombocytopenic purpura after pneumococcal vaccination.肺炎球菌疫苗接种后发生急性血栓性血小板减少性紫癜

Blood Coagul Fibrinolysis. 2014 Jul;25(5):512-4. doi: 10.1097/MBC.0000000000000058.

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Pulmonary involvement in patients with thrombotic thrombocytopenic purpura.血栓性血小板减少性紫癜患者的肺部受累。

Eur J Haematol. 2014 Feb;92(2):156-63. doi: 10.1111/ejh.12222. Epub 2013 Nov 26.

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Children and adults with thrombotic thrombocytopenic purpura associated with severe, acquired Adamts13 deficiency: comparison of incidence, demographic and clinical features.与严重获得性 ADAMTS13 缺乏相关的血栓性血小板减少性紫癜的儿童和成人：发病率、人口统计学和临床特征比较。

Pediatr Blood Cancer. 2013 Oct;60(10):1676-82. doi: 10.1002/pbc.24612. Epub 2013 Jun 1.

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Clinical features of severe acquired ADAMTS13 deficiency in thrombotic thrombocytopenic purpura: the Korean TTP registry experience.血栓性血小板减少性紫癜中获得性 ADAMTS13 缺乏症的临床特征：韩国 TTP 登记处的经验。

Int J Hematol. 2011 Feb;93(2):163-169. doi: 10.1007/s12185-011-0771-5. Epub 2011 Feb 3.

获得性血栓性血小板减少性紫癜，可能与阿斯利康-牛津新冠疫苗有关。

Acquired thrombotic thrombocytopenic purpura with possible association with AstraZeneca-Oxford COVID-19 vaccine.

作者信息

Al-Ahmad Mona, Al-Rasheed Mona, Shalaby Neveen Abo Bakr

机构信息

Microbiology Department Faculty of Medicine Kuwait University Kuwait Kuwait.

Al Rashed Allergy Centre Ministry of Health Kuwait.

出版信息

EJHaem. 2021 May 18;2(3):534-536. doi: 10.1002/jha2.219. eCollection 2021 Aug.

DOI:10.1002/jha2.219

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8242544/

Abstract

Acquired thrombotic thrombocytopenic purpura is characterized by the microvascular aggregation of platelets and microangiopathic hemolytic anemia causing ischemia of multiple organs including the brain mainly and less likely the kidney and the heart. The disease is caused by severe reduction in the activity of ADAMTS 13 due to presence of inhibitory antibodies.

摘要

获得性血栓性血小板减少性紫癜的特征是血小板在微血管聚集以及微血管病性溶血性贫血，导致包括脑在内的多个器官缺血，主要是脑，肾脏和心脏受累的可能性较小。该疾病是由于存在抑制性抗体导致ADAMTS 13活性严重降低所致。