Validation of the PLASMIC score for predicting ADAMTS13 activity < 10% in patients with suspected thrombotic thrombocytopenic purpura in Alberta, Canada
Validation of the PLASMIC score for predicting ADAMTS13 activity < 10% in patients with suspected thrombotic thrombocytopenic purpura in Alberta, Canada
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DOI：
10.1016/j.thromres.2020.09.012
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发表时间：
2020-12-01
影响因子：
7.5
通讯作者：
Sun, Haowei (Linda)
中科院分区：
文献类型：
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作者：
Wynick, Chris;Britto, Joanne;Sun, Haowei (Linda)
背景：血栓性血小板减少性紫癜（TTP）是一种危及生命的血栓性微血管病（TMA），需要立即进行血浆置换。临床预测工具可以促进周转时间延迟或 ADAMTS13 检测访问受限的机构的决策。 PLASMIC 评分和 Bentley 评分已被证明可以预测严重的 ADAMTS13 缺陷，具有出色的敏感性和特异性。 目的：使用疑似 TTP 人群验证 PLASMIC 评分，并与 Bentley 评分和临床格式塔相比，评估其预测严重 ADAMTS13 缺陷的辨别能力。方法：纳入了 2008 年至 2018 年间加拿大艾伯塔省疑似 TTP 且具有 ADAMTS13 结果的成年人。计算 PLASMIC 评分、Bentley 评分和临床格式塔的敏感性、特异性、阳性预测值 (PPV) 和阴性预测值 (NPV)。接收者操作员特征分析评估了评分系统的性能。结果：在 163 名疑似 TTP 的个体中，117 名 (72%) 存在 ADAMTS13 活性。 62 名患者 (53%) 中存在 10% 的严重 ADAMTS13 缺乏症。高风险 PLASMIC 评分 (= 20) 具有较低的敏感性 (59.5%) 和较高的特异性 (93.9%)，且具有相似的 c 统计量 (0.77)。临床格式塔与 PLASMIC 评分具有相似的敏感性，但特异性非常低 (16.1%)。 结论：与临床格式塔相比，PLASMIC 和 Bentley 评分在识别加拿大 TMA 人群中严重 ADAMTS13 缺陷方面具有良好的区分能力。融入机构临床路径可能有助于补充临床判断并降低成本。
Background: Thrombotic thrombocytopenic purpura (TTP) is a life-threatening thrombotic microangiopathy (TMA) that requires prompt plasma exchange. Clinical prediction tools may facilitate decision-making in institutions with delayed turnaround time or limited access to ADAMTS13 assays. The PLASMIC score and Bentley score have been shown to predict severe ADAMTS13 deficiency with excellent sensitivity and specificity.Objectives: To validate the PLASMIC score using a population of suspected TTP, and evaluate its discriminatory power in predicting severe ADAMTS13 deficiency in comparison with Bentley score and clinical gestalt. Methods: Adults presenting with suspected TTP in Alberta, Canada between 2008 and 2018 with available ADAMTS13 results were included. The sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) were calculated for PLASMIC score, Bentley score and clinical gestalt. Receiver operator characteristics analysis assessed the performance of the scoring systems.Results: Among 163 individuals with suspected TTP, ADAMTS13 activity was available in 117 (72%). Severe ADAMTS13 deficiency 10% was present in 62 (53%). High-risk PLASMIC score (= 20) had a lower sensitivity (59.5%) and higher specificity (93.9%) with similar c-statistic (0.77). Clinical gestalt had similar sensitivity as PLASMIC score but very low specificity (16.1%).Conclusions: Both PLASMIC and Bentley scores had good discriminatory power in identifying severe ADAMTS13 deficiency in a Canadian TMA population compared to clinical gestalt. Integration into institutional clinical pathways may help supplement clinical judgment and reduce costs.