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Validation of the ISTH/SSC bleeding assessment tool for inherited platelet disorders: a communication from the Platelet Physiology SSC
Article
Authorship:
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 ; SANCHEZ LUCEROS, ANALIA 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.Date:
2019Publishing House and Editing Place:
WILEY-BLACKWELL PUBLISHING, INCMagazine:
JOURNAL OF THROMBOSIS AND HAEMOSTASIS, vol. 18 (pp. 732-739) WILEY-BLACKWELL PUBLISHING, INCSummary
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.Key Words
INHERITED PLATELET DISORDERSBLEEDING DISORDERSBLEEDING DIATHESISBLEEDING ASSESSMENT TOOLPLATELETS