[Information on von Willebrand disease]

Area of Science:

• Hematology
• Clinical diagnostics
• Molecular biology

Context:

• The International Society of Thrombosis and Haemostasis (ISTH) established a VWD classification in 1993.
• VWD classification includes quantitative (Type 1), qualitative (Type 2), and deficiency (Type 3) defects.
• Type 2 VWD has subtypes based on VWF-platelet or VWF-FVIII binding.
• Diagnostic criteria for VWD are currently under review by the ISTH.

Purpose:

• To introduce updated diagnostic guidelines for von Willebrand disease (VWD) Type 1 and Type 2N.
• To address the ongoing reconsideration of VWD diagnostic criteria by the ISTH.
• To standardize the diagnosis of specific VWD subtypes within the department.

Summary:

• The 1993 ISTH classification categorizes VWD into Types 1, 2, and 3.
• Type 2 VWD encompasses subtypes 2A, 2B, 2M, and 2N, defined by specific binding defects.
• This work presents new departmental guidelines for diagnosing VWD Type 1 and Type 2N.

Impact:

• Facilitates more accurate and consistent diagnosis of VWD Type 1 and Type 2N.
• Contributes to the refinement of VWD diagnostic criteria.
• Improves patient management through precise VWD subtyping.