TY - JOUR
T1 - Validation of PLASMIC score and follow-up data in a cohort of patients with suspected microangiopathies from Southern Italy
AU - Tiscia, Giovanni Luca
AU - Ostuni, Angelo
AU - Cascavilla, Nicola
AU - Cappucci, Filomena
AU - Scalzulli, Potito
AU - Battista, Cosima
AU - Abrescia, Antonio
AU - Aucella, Filippo
AU - Buquicchio, Caterina
AU - Brigante, Maurizio
AU - D’Andrea, Giovanna
AU - Di Paolo, Bruno
AU - Giordano, Giulio
AU - Infante, Barbara
AU - Piano, Silvia
AU - Ranieri, Prudenza
AU - Tullo, Livio
AU - Grandone, Elvira
PY - 2018/8/1
Y1 - 2018/8/1
N2 - 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.
AB - 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.
KW - ADAMTS13
KW - Plasmapheresis
KW - Thrombotic microangiopathies
KW - Thrombotic thrombocytopenic purpura
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U2 - 10.1007/s11239-018-1674-6
DO - 10.1007/s11239-018-1674-6
M3 - Article
C2 - 29737462
AN - SCOPUS:85046669070
VL - 46
SP - 174
EP - 179
JO - Journal of Thrombosis and Thrombolysis
JF - Journal of Thrombosis and Thrombolysis
SN - 0929-5305
IS - 2
ER -