Browsing by Author "Rosa Cid, Ana"

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    Validation of the ISTH/SSC bleeding assessment tool for inherited platelet disorders: A communication from the Platelet Physiology SSC
    (2020) Gresele, Paolo; Orsini, Sara; Noris, Patrizia; Falcinelli, Emanuela; Alessi, Marie Christine; Bury, Loredana; Borhany, Munira; Santoro, Cristina; Glembotsky, Ana C.; Rosa Cid, Ana; Tosetto, Alberto; De Candia, Erica; Fontana, Pierre; Guglielmini, Giuseppe; Pecci, Alessandro; Melazzini, Federica; Falaise, Celine; 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., III; Harrison, Paul; Mezzano, Diego; Mumford, Andrew D.
    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.