info:eu-repo/semantics/article
Validation of the ISTH/SSC bleeding assessment tool for inherited platelet disorders: a communication from the Platelet Physiology SSC
Fecha
2019-12Registro en:
Gresele, Paolo; Orsini, Sara; Noris, Patrizia; Falcinelli, Emanuela; Alessi, Marie Christine; et al.; Validation of the ISTH/SSC bleeding assessment tool for inherited platelet disorders: a communication from the Platelet Physiology SSC; Wiley Blackwell Publishing, Inc; Journal of Thrombosis and Haemostasis; 18; 3; 12-2019; 732-739
1538-7933
CONICET Digital
CONICET
Autor
Gresele, Paolo
Orsini, Sara
Noris, Patrizia
Falcinelli, Emanuela
Alessi, Marie Christine
Bury, Loredana
Borhany, Munira
Santoro, Cristina
Glembotsky, Ana Claudia
Cid, Ana Rosa
Tosetto, Alberto
De Candia, Erica
Fontana, Pierre
Guglielmini, Giuseppe
Pecci, Alessandro
Heller, Paula Graciela
Rodorigo, Giuseppina
Lammle, Bernhard
Trinchero, Alice
Paolo, Radossi
Ferrari, Silvia
Rancitelli, Davide
Stolinski, Amy
Arulselvan, Abinaya
Lassandro, Giuseppe
Sánchez Luceros, Analía Gabriela
Jandrot Perrus, Martine
Kunishima, Shinji
Rivera Pozo, José
Lordkipanidzé, Marie
Melazzini, Federica
Falaise, Céline
Casonato, Alessandra
Podda, Gianmarco
Kannan, Meganathan
Jurk, Kerstin
Sevivas, Teresa
Castaman, Giancarlo
Grandone, Elvira
Fiore, Mathieu
Zuniga, Pamela
Henskens, Yvonne
Miyazaki, Koji
Dupuis, Arnaud
Hayward, Catherine
Zaninetti, Carlo
Abid, Madiha
Ferrara, Grazia
Mazzucconi, Maria Gabriella
Tagariello, Giuseppe
James, Paula
Fabris, Fabrizio
Russo, Alexandra
Bermejo, Nuria
Napolitano, Mariasanta
Curnow, Jennifer
Vasiliki, Gkalea
Zieger, Barbara
Fedor, Marian
Chitlur, Meera
Lambert, Michele
Barcella, Luca
Cosmi, Benilde
Giordano, Paola
Porri, Claudia
Eker, Ibrahim
Morel Kopp, Marie Christine
Deckmyn, Hans
Frelinger, Andrew L.
Harrison, Paul
Mezzano, Diego
Mumford, Andrew D.
Resumen
Background: Careful assessment of bleeding history is the first step in the evaluation of patients with mild/moderate bleeding disorders, and the use of a bleeding assessment tool (BAT) is strongly encouraged. Although a few studies have assessed the utility of the ISTH-BAT in patients with inherited platelet function disorders (IPFD) none of them was sufficiently large to draw conclusions and/or included appropriate control groups. Objectives: The aim of the present study was to test the utility of the ISTH-BAT in a large cohort of patients with a well-defined diagnosis of inherited platelets disorder in comparison with two parallel cohorts, one of patients with type-1 von Willebrand disease (VWD-1) and one of healthy controls (HC). Patients/Methods: We enrolled 1098 subjects, 482 of whom had inherited platelet disorders (196 IPFD and 286 inherited platelet number disorders [IT]) from 17 countries. Results: IPFD patients had significantly higher bleeding score (BS; median 9) than VWD-1 patients (median 5), a higher number of hemorrhagic symptoms (4 versus 3), and higher percentage of patients with clinically relevant symptoms (score > 2). The ISTH-BAT showed excellent discrimination power between IPFD and HC (0.9 < area under the curve [AUC] < 1), moderate (0.7 < AUC < 0.9) between IPFD and VWD-1 and between IPFD and inherited thrombocytopenia (IT), while it was inaccurate (AUC ≤ 0.7) in discriminating IT from HC. Conclusions: The ISTH-BAT allows to efficiently discriminate IPFD from HC, while it has lower accuracy in distinguishing IPFD from VWD-1. Therefore, the ISTH-BAT appears useful for identifying subjects requiring laboratory evaluation for a suspected IPFD once VWD is preliminarily excluded.