Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study
Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study
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DOI：
10.1016/s2352-3026(17)30026-1
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发表时间：
2017-04-01
影响因子：
24.7
通讯作者：
Makar, Robert S.
中科院分区：
文献类型：
--
作者：
Bendapudi, Pavan K.;Hurwitz, Shelley;Makar, Robert S.
背景在以血栓性微血管病为特征的综合征中，血栓性血小板减少性紫癜以ADAMTS13酶的严重缺乏为特征。患有这种疾病的患者需要紧急血浆置换治疗。由于ADAMTS13活性检测通常需要较长的周转时间，并且在资源贫乏的环境中可能无法获得，因此需要一种快速评估ADAMTS13严重缺乏的可能性的方法。和2015年12月6日，被纳入正在进行的多机构登记（哈佛TMA研究协作）。单变量分析用于确定logistic回归模型预测ADAMTS13严重缺陷的协变量（
Background Among the syndromes characterised by thrombotic microangiopathy, thrombotic thrombocytopenic purpura is distinguished by a severe deficiency in the ADAMTS13 enzyme. Patients with this disorder need urgent treatment with plasma exchange. Because ADAMTS13 activity testing typically requires prolonged turnaround times and might be unavailable in resource-poor settings, a method to rapidly assess the likelihood of severe ADAMTS13 deficiency is needed.Methods All consecutive adult patients presenting to three large academic medical centres in Boston, MA, USA, with thrombotic microangiopathy and a possible diagnosis of thrombotic thrombocytopenic purpura between Jan 8, 2004, and Dec 6, 2015, were included in an ongoing multi-institutional registry (the Harvard TMA Research Collaborative). Univariate analysis was used to identify covariates for a logistic regression model predictive of severe ADAMTS13 deficiency (