Von Willebrand Disease (VWD)
Part 2: NHLBI Diagnosis Guidelines

VWD Classification — ISTH^1,2

May 2011

VWD is classified on the basis of criteria developed by the VWF Subcommittee of the International Society on Thrombosis and Hemostasis (ISTH) in 1994 and revised in 2006.

VWD is classified into 3 major categories: Type 1 VWD reflecting partial quantitative VWF deficiency; Type 2 VWD reflecting qualitative VWF deficiency; and Type 3 VWD defined as total VWF deficiency. Type 1 VWD is the most common form and is of variable severity, whereas type 3 VWD is rare. Type 2 VWD has 4 variants or subtypes: Type 2A VWD, the most common, has decreased VWF-dependent platelet adhesion caused by deficiency of the high molecular weight multimers (HMWM) of VWF; Type 2B VWD reflects increased affinity of VWF for platelet GPIb, resulting in deficiency of VWF high molecular weight multimers, and is often accompanied by thrombocytopenia; Type 2M VWD has decreased VWF-dependent platelet adhesion NOT caused by deficiency of high molecular weight multimers; and Type 2N VWD reflects markedly decreased VWF binding affinity for factor VIII (FVIII).

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Jump to section:

• Introduction
• Objectives—Part 2
• VWD NHLBI Guidelines (2008)¹
• VWD Classification — ISTH^1,2
• VWD NHLBI Guidelines (2008) Initial Patient Evaluation — Laboratory¹
• VWD NHLBI Guidelines 2008 Initial Patient Evaluation — Laboratory¹
• VWD — Initial Laboratory Evaluation
• VWD NHLBI Guidelines (2008) Subsequent Patient Evaluation—Laboratory¹
• Additional VWD Testing — Laboratory¹
• VWF Multimer Analysis: Intermediate Resolution³
• VWF Multimer Analysis: Recommendations⁴
• VWF Multimer Analysis: Recommendations⁴
• Expected Laboratory Values in VWD¹
• Typical Patterns of Laboratory Test Results for VWD Diagnosis and Classification¹
• VWD NHLBI Guidelines (2008) "Low VWF" versus "VWD Type 1"
• VWD Diagnosis (Especially Type 1)
• VWD NHLBI Guidelines ABO Blood Group & VWF/VWD^1,5
• VWD NHLBI Guidelines ABO Blood Group and VWF/VWD: Soft Recommendations
• VWD NHLBI Guidelines (2008): Collection and Handling of Samples for Laboratory Testing¹
• VWD NHLBI Guidelines (2008): Collection and Handling of Samples for Laboratory Testing¹
• VWD NHLBI Guidelines (2008): Collection and Handling of Samples for Laboratory Testing¹
• VWD NHLBI Guidelines: Collection and Handling of Samples for Laboratory Testing^1,4
• VWD NHLBI Guidelines (2008) Summary of Laboratory Recommendations
• VWD NHLBI Guidelines (2008) Summary of Laboratory Recommendations
• References
• VWD Profile
• Questions?