要点国际合作为临床定义提供了共识。这涉及血栓性微血管病变和血栓性血小板减少性紫癜(TTP)。共识定义了诊断,疾病监测和对治疗的反应。给出了ADAMTS-13的要求。背景技术血栓性血小板减少性紫癜(TTP)和溶血性尿毒综合征(HUS)是需要诊断的两种重要急性病症。血栓性微血管病(TMA)是一种广泛的病理生理过程,可导致微血管病性溶血性贫血和血小板减少,并涉及毛细血管和小血管血小板聚集物。最常见的原因是弥散性血管内凝血,这可以通过异常凝血来区分。临床上,微血管病性溶血性贫血和血小板减少症,包括癌症,感染,移植,吸毒,自身免疫性疾病,先兆子痫和溶血,妊娠期肝酶升高和血小板计数低综合征。尽管临床表现重叠,TTP和HUS具有不同的病理生理学和治疗途径。目的介绍TTP和相关血栓性微血管病(TMA)国际工作组的共识文件。方法国际工作组根据已发布的信息和基于共识的建议提出了定义和术语。结论共识旨在帮助临床决策,还有未来的研究和试验,利用标准化的定义。它对TMA的原因进行了分类,和临床反应标准,先天性和免疫介导的TTP的缓解和复发。
Essentials An international collaboration provides a
consensus for clinical definitions. This concerns thrombotic microangiopathies and thrombotic thrombocytopenic purpura (TTP). The
consensus defines diagnosis, disease monitoring and response to treatment. Requirements for ADAMTS-13 are given.
Background Thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS) are two important acute conditions to diagnose. Thrombotic microangiopathy (TMA) is a broad pathophysiologic process that leads to microangiopathic hemolytic anemia and thrombocytopenia, and involves capillary and small-vessel platelet aggregates. The most common cause is disseminated intravascular coagulation, which may be differentiated by abnormal coagulation. Clinically, a number of conditions present with microangiopathic hemolytic anemia and thrombocytopenia, including cancer, infection, transplantation, drug use, autoimmune disease, and pre-eclampsia and hemolysis, elevated liver enzymes and low platelet count syndrome in pregnancy. Despite overlapping clinical presentations, TTP and HUS have distinct pathophysiologies and treatment pathways. Objectives To present a
consensus document from an International Working Group on TTP and associated thrombotic microangiopathies (TMAs). Methods The International Working Group has proposed definitions and terminology based on published information and
consensus-based recommendations. Conclusion The
consensus aims to aid clinical decisions, but also future studies and trials, utilizing standardized definitions. It presents a classification of the causes of TMA, and criteria for clinical response, remission and relapse of congenital and immune-mediated TTP.