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

Giovanni Luca Tiscia, Angelo Ostuni, Nicola Cascavilla, Filomena Cappucci, Potito Scalzulli, Cosima Battista, Antonio Abrescia, Filippo Aucella, Caterina Buquicchio, Maurizio Brigante, Giovanna D’Andrea, Bruno Di Paolo, Giulio Giordano, Barbara Infante, Silvia Piano, Prudenza Ranieri, Livio Tullo, Elvira Grandone

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)174-179
Number of pages6
JournalJournal of Thrombosis and Thrombolysis
Volume46
Issue number2
DOIs
Publication statusPublished - Aug 1 2018

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Keywords

  • ADAMTS13
  • Plasmapheresis
  • Thrombotic microangiopathies
  • Thrombotic thrombocytopenic purpura

ASJC Scopus subject areas

  • Hematology
  • Cardiology and Cardiovascular Medicine

Cite this

Tiscia, G. L., Ostuni, A., Cascavilla, N., Cappucci, F., Scalzulli, P., Battista, C., Abrescia, A., Aucella, F., Buquicchio, C., Brigante, M., D’Andrea, G., Di Paolo, B., Giordano, G., Infante, B., Piano, S., Ranieri, P., Tullo, L., & Grandone, E. (2018). Validation of PLASMIC score and follow-up data in a cohort of patients with suspected microangiopathies from Southern Italy. Journal of Thrombosis and Thrombolysis, 46(2), 174-179. https://doi.org/10.1007/s11239-018-1674-6