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Abstract：
The BENTLEY score (B-S), French thrombotic microangiopathy (TMA) Reference Center score (FTMA-S), and PLASMIC score (PLASMIC-S) have been developed for TMA diagnostic prediction. We retrospectively validated their predictive performances in patients with severe (<10%) disintegrin and metalloprotease with thrombospondin type 1 motif, member 13 (ADAMTS13) deficiency in terms of the risk of TMA and response to therapeutic plasma exchange (TPE).
The predictive performances of the three scoring systems were compared in 145 patients with suspected TMA who underwent ADAMTS13 activity tests between January 2014 and September 2022. The response to TPE and mortality in TMA-positive patients were compared after risk stratification, using the Mann-Whitney U and Fisher\'s exact tests.
The PLASMIC-S, FTMA-S, and B-S showed area under the curve values of 0.820, 0.636, and 0.513, respectively, for predicting TMA positivity in high-risk patients. The PLASMIC-S showed higher sensitivity (81.8%), negative predictive value (91.2%), positive predictive value (PPV; 66.7%), and accuracy (82.1%) than the FTMA-S (72.7%, 82.1%, 41.0%, and 60.0%, respectively) and B-S (4.6%, 70.2%, 50.0%, and 69.7%, respectively). The PLASMIC-S also showed higher specificity than the FTMA-S (82.2% vs. 54.5%). The modified PLASMIC-S, including lactate dehydrogenase/upper limit of normal ratios, increased the specificity, PPV, and accuracy to 97.0%, 92.3%, and 92.4%, respectively. In TMA-positive patients, high risk assessed by the PLASMIC-S predicted higher platelet recovery rates and less TPE sessions required for recovery than for those assessed at low-to-intermediate risk.
PLASMIC-S is the preferred scoring system for detecting patients with TMA positivity and for prognosis before confirmation of ADAMTS13 activity.
The predictive performances of the three scoring systems were compared in 145 patients with suspected TMA who underwent ADAMTS13 activity tests between January 2014 and September 2022. The response to TPE and mortality in TMA-positive patients were compared after risk stratification, using the Mann-Whitney U and Fisher\'s exact tests.
The PLASMIC-S, FTMA-S, and B-S showed area under the curve values of 0.820, 0.636, and 0.513, respectively, for predicting TMA positivity in high-risk patients. The PLASMIC-S showed higher sensitivity (81.8%), negative predictive value (91.2%), positive predictive value (PPV; 66.7%), and accuracy (82.1%) than the FTMA-S (72.7%, 82.1%, 41.0%, and 60.0%, respectively) and B-S (4.6%, 70.2%, 50.0%, and 69.7%, respectively). The PLASMIC-S also showed higher specificity than the FTMA-S (82.2% vs. 54.5%). The modified PLASMIC-S, including lactate dehydrogenase/upper limit of normal ratios, increased the specificity, PPV, and accuracy to 97.0%, 92.3%, and 92.4%, respectively. In TMA-positive patients, high risk assessed by the PLASMIC-S predicted higher platelet recovery rates and less TPE sessions required for recovery than for those assessed at low-to-intermediate risk.
PLASMIC-S is the preferred scoring system for detecting patients with TMA positivity and for prognosis before confirmation of ADAMTS13 activity.
摘要：
■本特利得分（B-S），法国血栓性微血管病（TMA）参考中心评分（FTMA-S），和PLASMIC评分（PLASMIC-S）已开发用于TMA诊断预测。我们回顾性地验证了他们在严重（<10％）的崩解素和金属蛋白酶与血小板反应蛋白1型基序的患者中的预测性能，成员13（ADAMTS13）在TMA风险和治疗性血浆置换（TPE）反应方面的缺陷。
■在2014年1月至2022年9月期间接受ADAMTS13活动测试的145例疑似TMA患者中，比较了三种评分系统的预测性能。TMA阳性患者对TPE的反应和死亡率在危险分层后进行比较，使用曼-惠特尼U和费舍尔的精确检验。
■等离子体-S,FTMA-S,和B-S显示曲线下面积值分别为0.820、0.636和0.513，用于预测高危患者的TMA阳性。PLASMIC-S显示出更高的灵敏度（81.8%），阴性预测值（91.2%），阳性预测值（PPV；66.7%），和准确性（82.1%）比FTMA-S（72.7%，82.1%,41.0%,和60.0%，分别)和B-S(4.6%，70.2%,50.0%,和69.7%，分别)。PLASMIC-S也显示出比FTMA-S更高的特异性(82.2%vs.54.5%）。改性的PLASMIC-S,包括乳酸脱氢酶/正常比率上限，增加了特异性，PPV,准确度达到97.0%，92.3%,92.4%,分别。在TMA阳性患者中，PLASMIC-S评估的高风险预测,与低至中风险评估者相比,血小板恢复率较高,恢复所需的TPE疗程较少.
■PLASMIC-S是检测TMA阳性患者和确认ADAMTS13活性前预后的首选评分系统。
■在2014年1月至2022年9月期间接受ADAMTS13活动测试的145例疑似TMA患者中，比较了三种评分系统的预测性能。TMA阳性患者对TPE的反应和死亡率在危险分层后进行比较，使用曼-惠特尼U和费舍尔的精确检验。
■等离子体-S,FTMA-S,和B-S显示曲线下面积值分别为0.820、0.636和0.513，用于预测高危患者的TMA阳性。PLASMIC-S显示出更高的灵敏度（81.8%），阴性预测值（91.2%），阳性预测值（PPV；66.7%），和准确性（82.1%）比FTMA-S（72.7%，82.1%,41.0%,和60.0%，分别)和B-S(4.6%，70.2%,50.0%,和69.7%，分别)。PLASMIC-S也显示出比FTMA-S更高的特异性(82.2%vs.54.5%）。改性的PLASMIC-S,包括乳酸脱氢酶/正常比率上限，增加了特异性，PPV,准确度达到97.0%，92.3%,92.4%,分别。在TMA阳性患者中，PLASMIC-S评估的高风险预测,与低至中风险评估者相比,血小板恢复率较高,恢复所需的TPE疗程较少.
■PLASMIC-S是检测TMA阳性患者和确认ADAMTS13活性前预后的首选评分系统。
