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

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dc.contributor.authorGresele, Paolo
dc.contributor.authorOrsini, Sara
dc.contributor.authorNoris, Patrizia
dc.contributor.authorFalcinelli, Emanuela
dc.contributor.authorAlessi, Marie Christine
dc.contributor.authorBury, Loredana
dc.contributor.authorBorhany, Munira
dc.contributor.authorSantoro, Cristina
dc.contributor.authorGlembotsky, Ana C.
dc.contributor.authorRosa Cid, Ana
dc.contributor.authorTosetto, Alberto
dc.contributor.authorDe Candia, Erica
dc.contributor.authorFontana, Pierre
dc.contributor.authorGuglielmini, Giuseppe
dc.contributor.authorPecci, Alessandro
dc.contributor.authorMelazzini, Federica
dc.contributor.authorFalaise, Celine
dc.contributor.authorCasonato, Alessandra
dc.contributor.authorPodda, Gianmarco
dc.contributor.authorKannan, Meganathan
dc.contributor.authorJurk, Kerstin
dc.contributor.authorSevivas, Teresa
dc.contributor.authorCastaman, Giancarlo
dc.contributor.authorGrandone, Elvira
dc.contributor.authorFiore, Mathieu
dc.contributor.authorZuniga, Pamela
dc.contributor.authorHenskens, Yvonne
dc.contributor.authorMiyazaki, Koji
dc.contributor.authorDupuis, Arnaud
dc.contributor.authorHayward, Catherine
dc.contributor.authorZaninetti, Carlo
dc.contributor.authorAbid, Madiha
dc.contributor.authorFerrara, Grazia
dc.contributor.authorMazzucconi, Maria Gabriella
dc.contributor.authorTagariello, Giuseppe
dc.contributor.authorJames, Paula
dc.contributor.authorFabris, Fabrizio
dc.contributor.authorRusso, Alexandra
dc.contributor.authorBermejo, Nuria
dc.contributor.authorNapolitano, Mariasanta
dc.contributor.authorCurnow, Jennifer
dc.contributor.authorVasiliki, Gkalea
dc.contributor.authorZieger, Barbara
dc.contributor.authorFedor, Marian
dc.contributor.authorChitlur, Meera
dc.contributor.authorLambert, Michele
dc.contributor.authorBarcella, Luca
dc.contributor.authorCosmi, Benilde
dc.contributor.authorGiordano, Paola
dc.contributor.authorPorri, Claudia
dc.contributor.authorEker, Ibrahim
dc.contributor.authorMorel-Kopp, Marie-Christine
dc.contributor.authorDeckmyn, Hans
dc.contributor.authorFrelinger, Andrew L., III
dc.contributor.authorHarrison, Paul
dc.contributor.authorMezzano, Diego
dc.contributor.authorMumford, Andrew D.
dc.date.accessioned2025-01-23T19:48:34Z
dc.date.available2025-01-23T19:48:34Z
dc.date.issued2020
dc.description.abstractBackground 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.
dc.fuente.origenWOS
dc.identifier.doi10.1111/jth.14683
dc.identifier.eissn1538-7836
dc.identifier.issn1538-7933
dc.identifier.urihttps://doi.org/10.1111/jth.14683
dc.identifier.urihttps://repositorio.uc.cl/handle/11534/100450
dc.identifier.wosidWOS:000502794200001
dc.issue.numero3
dc.language.isoen
dc.pagina.final739
dc.pagina.inicio732
dc.revistaJournal of thrombosis and haemostasis
dc.rightsacceso restringido
dc.subjectbleeding assessment tool
dc.subjectinherited platelet disorders
dc.subjectbleeding diathesis
dc.subjectplatelets
dc.subjectbleeding disorders
dc.subject.ods03 Good Health and Well-being
dc.subject.odspa03 Salud y bienestar
dc.titleValidation of the ISTH/SSC bleeding assessment tool for inherited platelet disorders: A communication from the Platelet Physiology SSC
dc.typeartículo
dc.volumen18
sipa.indexWOS
sipa.trazabilidadWOS;2025-01-12
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