Shaw Rebecca J, Abrams Simon T, Badu Samuel, Toh Cheng-Hock, Dutt Tina
Liverpool University Hospitals NHS Foundation Trust, Liverpool L7 8YE, UK.
Clinical Infection, Microbiology and Immunology, Institute of Infection, Veterinary & Ecological Sciences, University of Liverpool, Ronald Ross Building, 8 West Derby Street, Liverpool L69 7BE, UK.
J Clin Med. 2024 Aug 30;13(17):5152. doi: 10.3390/jcm13175152.
Severe deficiency of ADAMTS13 (<10 iu/dL) is diagnostic of thrombotic thrombocytopenic purpura (TTP) and leads to accumulation of ultra-large vWF multimers, platelet aggregation, and widespread microthrombi, which can be life-threatening. However, the clinical implications of a low ADAMTS13 activity level are not only important in an acute episode of TTP. In this article, we discuss the effects of low ADAMTS13 activity in congenital and immune-mediated TTP patients not only at presentation but once in a clinical remission. Evidence is emerging of the clinical effects of low ADAMTS13 activity in other disease areas outside of TTP, and here, we explore the wider impact of low ADAMTS13 activity on the vascular endothelium and the potential for recombinant ADAMTS13 therapy in other thrombotic disease states.
ADAMTS13严重缺乏（<10 iu/dL）可诊断为血栓性血小板减少性紫癜（TTP），并导致超大vWF多聚体的积累、血小板聚集和广泛的微血栓形成，这可能危及生命。然而，低ADAMTS13活性水平的临床意义不仅在TTP急性发作时很重要。在本文中，我们不仅讨论了低ADAMTS13活性对先天性和免疫介导性TTP患者在疾病表现时的影响，还讨论了其在临床缓解期的影响。有证据表明，低ADAMTS13活性在TTP以外的其他疾病领域也有临床影响，在此，我们探讨低ADAMTS13活性对血管内皮的更广泛影响以及重组ADAMTS13治疗在其他血栓性疾病状态中的潜力。
