Validation of PLASMIC score and follow-up data in a cohort of patients with suspected microangiopathies from Southern Italy

Abstract

Severe ADAMTS13 deficiency (activity < 10%) is pathognomonic of thrombotic thrombocytopenic purpura. ADAMTS13 testing is time-consuming and unavailable in many hospitals. Recently, a seven-variables score named PLASMIC score, has been developed to stratify acute patients, based on their risk of having a severe ADAMTS13 deficiency. We present the application of this score in a cohort of patients referred to our Center. From 2012 to 2017, 42 patients with suspected thrombotic microangiopathies from 6 Centers were referred to Hemostasis and Thrombosis Center of "Casa Sollievo della Sofferenza" Hospital/Research Institute for ADAMTS13 testing. For all patients, relevant medical and laboratory information were collected. To obtain the statistical measure of the discriminatory power of PLASMIC scoring system, the Area Under the Curve Receiver Operating Characteristic (AUC ROC) was calculated. We were able to calculate the PLASMIC score in 27 out of 42 patients; we found a good discrimination performance of the score with a resulting AUC value of 0.86 (95% CI 0.71-1.0; p = 0.015). All patients but one with a high risk PLASMIC score (6-7) showed a severe deficiency. Among patients belonging to the intermediate risk (PLASMIC score 5) group, 2 showed normal ADAMTS13 activity and 2 levels below 10%. In none of the patients in the low risk group (PLASMIC score 0-4), a severe ADAMTS13 deficiency was found. Present results confirm and extend previous data regarding the predictive value of the PLASMIC score. Indeed, it shows a good diagnostic performance and can be useful for decision makers to properly and promptly define the better therapeutic approach.

Diagnostic accuracy of the PLASMIC score in patients with suspected thrombotic thrombocytopenic purpura: A systematic review and meta‐analysis

“…Twelve articles met eligibility criteria, one of which reported the results of 2 distinct studies, for a total of 13 eligible studies (Figure 1). 5,16‐26 …”

“…None of the studies was judged to be at high risk of bias with respect to the index test domain. However, concern regarding applicability to clinical practice was classified as high for multiple studies because the PLASMIC score was calculated by study personnel rather than a member of the patientʼs clinical team 5,17,18,21,25 …”

The Prognostic Value of ADAMTS-13 and von Willebrand Factor in COVID-19 Patients: Prospective Evaluation by Care Setting

“…VWF:RCo and VWF:Ag were measured, using an automated latex immunoassay in human citrated plasma on ACL TOP ® coagulation systems (HemosIL ® ). ADAMTS-13 activity was measured, using a chromogenic ELISA assay (TECHNOZYM ® ADAMTS-13 activity ELISA kit, Technoclone, Vienna, Austria) as previously described [ 12 ].…”

Rare Defects: Looking at the Dark Face of the Thrombosis

“…It may result in ischemic stroke in conjunction with additional genetic or environmental factors [97]. Considering the pivotal role of ADAMTS13 in the modulation of the coagulation cascade, a significant effect in the development of VTE has been suggested [98]. It was supposed that genetic factors affecting ADAMTS13 activity might modulate the risk for VTE by modifying VWF and FVIII levels.…”