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

Erica De Candia, Paolo Gresele, Sara Orsini, Patrizia Noris, Emanuela Falcinelli, Marie Christine Alessi, Loredana Bury, Munira Borhany, Cristina Santoro, Ana C. Glembotsky, Ana Rosa Cid, Alberto Tosetto, Pierre Fontana, Giuseppe Guglielmini, Alessandro Pecci, Paula G. Heller, Giuseppina Rodorigo, Bernhard Lammle, Alice Trinchero, Radossi PaoloSilvia Ferrari, Davide Rancitelli, Amy Stolinski, Abinaya Arulselvan, Giuseppe Lassandro, Analia Sanchez Luceros, Martine Jandrot-Perrus, Shinji Kunishima, José Rivera Pozo, Marie Lordkipanidzé, Federica Melazzini, Céline Falaise, Alessandra Casonato, Gianmarco Podda, Meganathan Kannan, Kerstin Jurk, Teresa Sevivas, Giancarlo Castaman, Elvira Grandone, Mathieu Fiore, Pamela Zuniga, Yvonne Henskens, Koji Miyazaki, Arnaud Dupuis, Catherine Hayward, Carlo Zaninetti, Madiha Abid, Grazia Ferrara, Maria Gabriella Mazzucconi, Giuseppe Tagariello, Paula James, Fabrizio Fabris, Alexandra Russo, Nuria Bermejo, Mariasanta Napolitano, Jennifer Curnow, Gkalea Vasiliki, Barbara Zieger, Marian Fedor, Meera Chitlur, Michele Lambert, Luca Barcella, Benilde Cosmi, Paola Giordano, Claudia Porri, Ibrahim Eker, Marie-Christine Morel-Kopp, Hans Deckmyn, Andrew L. Frelinger, Paul Harrison, Diego Mezzano, Andrew D. Mumford

Research output: Contribution to journalArticle

16 Citations (Scopus)

Abstract

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.
Original languageEnglish
Pages (from-to)732-739
Number of pages8
JournalJournal of Thrombosis and Haemostasis
Volume18
DOIs
Publication statusPublished - 2020

Keywords

  • bleeding assessment tool
  • bleeding diathesis
  • bleeding disorders
  • inherited platelet disorders
  • platelets

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