Bleeding assessment tools to predict von Willebrand disease: Utility of individual bleeding symptoms

Abstract

This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made. AbstractBackground: Bleeding assessment is part of the diagnostic workup of von Willebrand disease (VWD). Bleeding assessment tools (BATs) have standardized obtaining this information but have been criticized because they are time consuming.Objective: To use our legacy data to determine which questions from BATs are the strongest predictors of a VWD diagnosis.Patients/Methods: Bleeding score data from 3 different BATs were used. Patients aged <12 years were excluded. Questions on BATs relate to different bleeding symptoms, and each symptom is scored by severity. Scores for each symptom were sorted based on whether they indicated clinically significant bleeding, and nonsignificant scores were set as the reference category. Multivariable logistic regression was used to determine the symptoms that were the strongest predictors of a laboratory-confirmed VWD diagnosis. Results:A total of 927 participants were included; 144 (16%) were patients with VWD, and 783 (84%) were healthy controls. The top 3 symptoms for which a clinically significant positive response increased the likelihood of VWD were hemarthrosis (odds ratio [OR], 19.2; 95% confidence interval [CI], 3.7-100.4), postsurgical bleeding (OR, 15.2; 95% CI, 5.9-38.9), and menorrhagia (OR, 10.3; 95% CI, 4.9-21.9).With each increase in number of bleeding symptom categories with clinically significant scores, subjects had a stepwise increase in odds of a VWD diagnosis. Conclusions:Our results suggest that most of the bleeding symptoms on BATs are significant predictors of VWD, and there is value in assessing multiple bleeding symptoms when eliciting a bleeding history. Certain bleeding symptoms are more useful predictors than others. Future BAT revisions may consider adding a relative weighting to each symptom.

Obstacles to Early Diagnosis and Treatment of Inherited von Willebrand Disease: Current Perspectives

“… 45 BAT is an important mean to gather a detailed and standardized bleeding history thus selecting patients requiring additional hemostatic evaluation. 46 …”

An illustrated review of bleeding assessment tools and common coagulation tests

“…In addi on, the number of bleeding symptoms increased the odds ra o for a VWD diagnosis. [16] Bleeding symptom categories in the ISTH BAT Bleeding after hemostatic challenges:…”

Perioperative management for patients with von Willebrand disease: Defining the optimal approach

“…Optimal surgical management in patients with VWD is dependent on multiple factors including the type of surgery, type of VWD, baseline VWF and FVIII levels, and the individual patient's history of bleeding. Bleeding assessment tools have been developed as a quantitative measurement to rate bleeding symptoms, disease severity and family history of bleeding, whereby the preoperative “bleeding score” can be used to predict postoperative bleeding outcomes 16,17 . Preoperative scores range from −1 (absence of bleeding after significant haemostatic challenge such as two dental extractions or surgeries) to 4 (symptoms requiring the most significant medical intervention such as infusion of clotting factor concentrates or surgery to control bleeding); this approach can help reduce bleed complications by promoting timely interventions 16,17 .…”

“…Bleeding assessment tools have been developed as a quantitative measurement to rate bleeding symptoms, disease severity and family history of bleeding, whereby the preoperative “bleeding score” can be used to predict postoperative bleeding outcomes 16,17 . Preoperative scores range from −1 (absence of bleeding after significant haemostatic challenge such as two dental extractions or surgeries) to 4 (symptoms requiring the most significant medical intervention such as infusion of clotting factor concentrates or surgery to control bleeding); this approach can help reduce bleed complications by promoting timely interventions 16,17 . Currently, consensus on the preferred surgical management for all patients is lacking and many of the recommendations regarding the use of replacement therapy are based primarily on expert opinion 15 …”