The ISTH bleeding assessment tool as predictor of bleeding events in inherited platelet disorders: Communication from the ISTH SSC Subcommittee on Platelet Physiology Academic Article uri icon

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  • Background

    The ISTH Bleeding Assessment Tool (ISTH-BAT) has been validated for clinical screening of suspected von Willebrand disease (VWD) and for bleeding prediction. Recently it has been validated for subjects with inherited platelet disorders (IPD) (BAT-VAL study).


    To determine whether the ISTH-BAT bleeding score (BS) predicts subsequent bleeding events requiring treatment in IPD patients.


    Patients with IPD, type 1 VWD (VWD-1) and age- and sex-matched healthy controls enrolled in the BAT-VAL study were prospectively followed-up for 2 years and bleeding episodes requiring treatment were recorded.


    Of the 1098 subjects initially enrolled, 955 were followed-up and 124 suffered hemorrhages during follow-up, 60% of whom had inherited platelet function disorders (IPFD). Total number of events was significantly higher in IPFD (n = 235) than VWD-1 (n = 52) or inherited thrombocytopenia (IT; n = 20). Events requiring transfusions were 66% in IPFD, 5.7% in VWD-1, and 3% in IT. Baseline BS was significantly higher in IPFD patients with a bleeding event at follow-up than in those without (p < .01) and the percentage of subjects suffering a bleeding event increased proportionally to baseline BS quartile. A significant association between the BS and the chance of suffering severe bleeding was found in the overall, IPFD, and VWD-1 populations. Similar results were obtained for the pediatric population.


    Inherited platelet function disorder patients with high BS at enrollment are more likely to suffer from bleeding events requiring treatment at follow-up. Moreover, the higher the baseline BS quartile the greater the incidence of subsequent events, suggesting that independently from diagnosis a high BS is associated with a greater risk of subsequent hemorrhage.


  • Gresele, Paolo
  • Falcinelli, Emanuela
  • Bury, Loredana
  • Pecci, Alessandro
  • Alessi, Marie‐Christine
  • Borhany, Munira
  • Heller, Paula G
  • Santoro, Cristina
  • Cid, Ana Rosa
  • Orsini, Sara
  • Fontana, Pierre
  • De Candia, Erica
  • Podda, Gianmarco
  • Kannan, Meganathan
  • Jurk, Kerstin
  • Castaman, Giancarlo
  • Falaise, Céline
  • Guglielmini, Giuseppe
  • Noris, Patrizia
  • Zaninetti, Carlo
  • Fiore, Mathieu
  • Tosetto, Alberto
  • Zuniga, Pamela
  • Miyazaki, Koji
  • Dupuis, Arnaud
  • Hayward, Catherine Pauline Ma
  • Casonato, Alessandra
  • Grandone, Elvira
  • Mazzucconi, Maria Gabriella
  • James, Paula
  • Fabris, Fabrizio
  • Henskens, Yvonne
  • Napolitano, Mariasanta
  • Curnow, Jennifer
  • Gkalea, Vasiliki
  • Fedor, Marian
  • Lambert, Michele P
  • Zieger, Barbara
  • Barcella, Luca
  • Cosmi, Benilde
  • Giordano, Paola
  • Porri, Claudia
  • Melazzini, Federica
  • Abid, Madiha
  • Glembotsky, Ana C
  • Ferrara, Grazia
  • Russo, Alexandra
  • Deckmyn, Hans
  • Frelinger, Andrew L
  • Harrison, Paul
  • Mezzano, Diego
  • Mumford, Andrew D
  • Lordkipanidzé, Marie

publication date

  • May 2021