Validation of the ISTH/SSC bleeding assessment tool for inherited platelet disorders: A communication from the Platelet Physiology SSC

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...

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Published in:	Journal of thrombosis and haemostasis 2020-03, Vol.18 (3), p.732-739
Main Authors:	Gresele, Paolo, Orsini, Sara, Noris, Patrizia, Falcinelli, Emanuela, Alessi, Marie Christine, Bury, Loredana, Borhany, Munira, Santoro, Cristina, Glembotsky, Ana C., Cid, Ana Rosa, Tosetto, Alberto, De Candia, Erica, Fontana, Pierre, Guglielmini, Giuseppe, Pecci, Alessandro, Heller, Paula G., Rodorigo, Giuseppina, Lammle, Bernhard, Trinchero, Alice, Paolo, Radossi, Ferrari, Silvia, Rancitelli, Davide, Stolinski, Amy, Arulselvan, Abinaya, Lassandro, Giuseppe, Luceros, Analia Sanchez, 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.
Format:	Article
Language:	eng
Subjects:	Bleeding bleeding assessment tool bleeding diathesis bleeding disorders Blood Platelet Disorders - diagnosis Blood Platelet Disorders - genetics Communication Hemorrhage Hemorrhage - diagnosis Humans inherited platelet disorders Platelet Function Tests Platelets Thrombocytopenia von Willebrand Diseases - diagnosis von Willebrand Diseases - genetics
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Summary:	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
ISSN:	1538-7933 1538-7836 1538-7836