Department of Clinical Medicine and Surgery, "Federico II" University, Naples, Italy.
Advanced Biochemistry Unit Medylab Institute, Lusciano (CE), Italy.
Blood Transfus. 2023 Jul;21(4):345-349. doi: 10.2450/2022.0082-22. Epub 2022 Aug 9.
The PLASMIC score is a rapid and inexpensive clinical assessment tool for predicting severe ADAMTS13 deficiency (<10% activity) in patients with suspected thrombotic thrombocytopenic purpura (TTP). The score includes 7 parameters: absence of active cancer, patient not having received stem cell transplant or organ transplant, platelet count <30×10/L, hemolysis, mean corpuscular volume <90 fl, International Normalized Ratio <1.5, and serum creatinine <2 mg/dL.
In this retrospective study, we evaluated a cohort of 59 consecutive patients with suspected thrombotic microangiopathy who had been referred to the Hemostasis and Thrombosis Center of the "Federico II" University of Naples, Italy, for measurement of ADAMTS13 activity. Relevant clinical and laboratory information were collected for all patients.
The PLASMIC score was calculated in 52 of the 59 patients included in the study. In the high-risk group (PLASMIC score 6 or 7), 12 out of 20 patients (60%) had ADAMTS13 <10%. Interestingly, all 6 patients (100%) with PLASMIC score 7 had ADAMTS13 <5%. In the intermediate risk group (score 5), only one case out of 17 (5.9%) had ADAMTS 13 <10%. In the low-risk group (score 0-4), none of the patients had severe ADAMTS13 deficiency. The collected data enabled the sensitivity and specificity of PLASMIC score in TTP to be calculated, achieving 92% (95% CI: 0.80-0.98) and 79% (95% CI: 0.66-0.89), respectively. The PLASMIC score was seen to be a very efficient tool in distinguishing between patients with severe ADAMTS13 deficiency from those without, with an AUC of 0.92 (95% CI: 0.82-1.0; p<0.001).
In our cohort, a high-risk PLASMIC score successfully predicted patients with severe ADAMTS13 deficiency, allowing the clinician to quickly define the best therapeutic approach, especially useful for those clinicians not used to the diagnosis and treatment of thrombotic microangiopathies.
PLASMIC 评分是一种快速且廉价的临床评估工具，可用于预测疑似血栓性血小板减少性紫癜（TTP）患者中严重 ADAMTS13 缺乏症（<10%活性）。该评分包括 7 个参数：无活动性癌症、患者未接受过干细胞移植或器官移植、血小板计数<30×10/L、溶血、平均红细胞体积<90fl、国际标准化比值<1.5 和血清肌酐<2mg/dL。
在这项回顾性研究中，我们评估了意大利那不勒斯“Federico II”大学止血和血栓形成中心收治的 59 例疑似血栓性微血管病患者队列，这些患者均接受 ADAMTS13 活性检测。所有患者均收集了相关的临床和实验室信息。
59 例患者中有 52 例计算了 PLASMIC 评分。在高危组（PLASMIC 评分 6 或 7）中，20 例患者中有 12 例（60%）ADAMTS13<10%。有趣的是，PLASMIC 评分 7 的 6 例患者（100%）ADAMTS13<5%。在中危组（评分 5）中，17 例患者中有 1 例（5.9%）ADAMTS13<10%。在低危组（评分 0-4）中，无患者出现严重 ADAMTS13 缺乏症。所收集的数据使我们能够计算 PLASMIC 评分在 TTP 中的灵敏度和特异性，分别达到 92%（95%CI：0.80-0.98）和 79%（95%CI：0.66-0.89）。PLASMIC 评分在区分严重 ADAMTS13 缺乏症患者与非缺乏症患者方面非常有效，AUC 为 0.92（95%CI：0.82-1.0；p<0.001）。
在我们的队列中，高危 PLASMIC 评分成功预测了严重 ADAMTS13 缺乏症患者，使临床医生能够快速确定最佳治疗方法，特别是对那些不熟悉血栓性微血管病诊断和治疗的临床医生非常有用。
