The utility of International Society on Thrombosis and Haemostasis-Bleeding Assessment Tool and other bleeding questionnaires in assessing the bleeding phenotype in two platelet function defects

Blood Coagul Fibrinolysis. 2016 Jul;27(5):589-93. doi: 10.1097/MBC.0000000000000496.

Abstract

The main objective of this study is to investigate the utility of International Society on Thrombosis and Haemostasis-Bleeding Assessment Tool (ISTH-BAT) in comparison with the condensed form of Molecular and Clinical Markers for the Diagnosis and Management of type 1 and WHO BATs, in assessing bleeding in two well known and clinically significant platelet function defects. Thirty-eight patients previously diagnosed with Glanzmann's thrombasthenia and 10 with Bernard-Soulier syndrome (BSS) were analyzed. Bleeding scores were significantly higher than that of controls using both electronic bleeding questionnaire (eBQ) and ISTH-BAT with no significant difference between both tools. ISTH-BAT had a sensitivity, specificity, positive predictive value and negative predictive value of 100%, 76.2%, 0.9 and 1. This was closely similar to eBQ. Both ISTH-BAT and eBQ are efficient in BSS and Glanzmann's thrombasthenia. However, given the ISTH recommendation, ISTH-BAT should be adopted. Larger study including other platelet defects will enhance its utility and support the integration of bleeding scores with standardized laboratory testing to allow for a universal diagnostic approach to patients with suspected bleeding disorders.

MeSH terms

  • Adolescent
  • Adult
  • Bernard-Soulier Syndrome / blood
  • Bernard-Soulier Syndrome / diagnosis*
  • Bernard-Soulier Syndrome / pathology
  • Blood Platelets / metabolism
  • Blood Platelets / pathology
  • Case-Control Studies
  • Child
  • Child, Preschool
  • Diagnostic Self Evaluation
  • Female
  • Hemorrhage / blood
  • Hemorrhage / diagnosis*
  • Hemorrhage / pathology
  • Humans
  • Infant
  • Male
  • Middle Aged
  • Surveys and Questionnaires
  • Thrombasthenia / blood
  • Thrombasthenia / diagnosis*
  • Thrombasthenia / pathology
  • Thrombosis / blood
  • Thrombosis / diagnosis*
  • Thrombosis / pathology