Longitudinal bleeding assessment in von Willebrand disease utilizing an interim bleeding score

Area of Science:

• Hematology
• Clinical Diagnostics
• Patient Monitoring

Background:

• Assessing bleeding phenotype is crucial for diagnosing von Willebrand disease (VWD).
• Standardized tools for evaluating interim bleeding (bleeding after diagnosis) are currently lacking.
• Existing bleeding assessment tools (BATs) require refinement for longitudinal monitoring.

Purpose of the Study:

• To evaluate the clinical utility of a novel interim bleeding protocol for patients with VWD.
• To establish a standardized method for assessing bleeding events between diagnoses.
• To gain insights into the evolution of bleeding patterns in VWD over time.

Main Methods:

• A multicenter, international study involving patients with VWD.
• Utilized the International Society on Thrombosis and Haemostasis BAT, adapted to capture only interval bleeding (Interim BS).
• Compared interim bleeding scores (1 BS) with original bleeding scores (0 BS) and analyzed annualized scores (BS/yr) by VWD subtype, VWF level, sex, and age.

Main Results:

• The interim bleeding score (1 BS) effectively discriminated by VWD subtype, showing significantly higher scores in type 3 VWD.
• In type 1 VWD, the initial bleeding score (0 BS) did not predict future bleeding, whereas the interim score (1 BS/yr) showed similar rates regardless of baseline score.
• Interim bleeding scores did not significantly differ between sexes or age groups in type 1 VWD, and were similar across varying plasma von Willebrand factor levels.

Conclusions:

• The developed interim bleeding protocol offers a new approach for longitudinal bleeding assessment in VWD.
• The study provides valuable insights into the natural evolution of bleeding in patients with VWD.
• This protocol can aid in more accurate monitoring and management of bleeding risks in VWD patients.