Thrombotic thrombocytopenic purpura: celebrating 25 years of ADAMTS13.

The discovery of ADAMTS13 has been fundamental to the vast improvement seen in TTP outcomes.

ADAMTS13的发现对于TTP治疗结果的巨大改善起到了根本性的作用。

Understanding the interaction between ADAMTS13, platelets, and von Willebrand factor led to the development of clinical ADAMTS13 assays, and therefore quicker and accurate diagnosis, but also, critically, to novel therapies and monitoring of treatment.

了解ADAMTS13、血小板和冯·威尔勃兰因子之间的相互作用，促进了临床ADAMTS13检测方法的发展，从而实现了更快、更准确的诊断，但同样关键的是，它还促进了新型疗法和治疗监测。

Landmark additions to iTTP therapy have included anti-CD20 treatment with rituximab, in both the acute and elective settings, and the use of the nanobody caplacizumab in acute TTP.

iTTP治疗的重要补充包括在急性期和选择性情况下使用抗CD20治疗利妥昔单抗，以及在急性TTP中使用纳米体卡普拉西珠单抗。

In cTTP, the use of ADAMTS13 replacement is playing a role in reducing end-organ damage and morbidity, with recombinant ADAMTS13 (rADAMTS13) now representing the gold standard for cTTP.

在获得性血小板减少性紫癜（cTTP）中，ADAMTS13替代疗法在减少终末器官损伤和发病率方面发挥着作用，重组ADAMTS13（rADAMTS13）现在已成为cTTP的金标准。

The ability to measure response to treatment by monitoring ADAMTS13 activity has underpinned these treatment advances, and allowed clinicians to tailor immunosuppressive treatment for iTTP and rADAMTS13 dosing in cTTP.

通过监测ADAMTS13活性来衡量对治疗的反应，这一能力支撑了这些治疗进展，并使临床医生能够为免疫性血小板减少性紫癜（iTTP）定制免疫抑制治疗和为获得性血小板减少性紫癜（cTTP）调整重组ADAMTS13（rADAMTS13）剂量。

Looking forward, there are many avenues for future development, with potential expansion of rADAMTS13 to treat iTTP, new, quicker assays to improve diagnosis, monitoring, and immunomodulatory therapeutic advancement, all underpinned by ADAMTS13.

展望未来，有许多发展途径，rADAMTS13治疗iTTP的潜在扩展，新的、更快的检测方法以改善诊断、监测和免疫调节治疗的进步，所有这些都以ADAMTS13为基础。

Future endeavors for the role of ADAMTS13 in other thrombotic indications open further exciting opportunities.

ADAMTS13在其他血栓性疾病的未来角色，开启了更多令人兴奋的机会。