Von Willebrand Factor (VWF) Level Test

von Willebrand Factor (VWF) Level Testing

Key Points

  • Essential for diagnosing von Willebrand Disease (VWD)
  • Comprises multiple assays for comprehensive assessment
  • Age and blood type influence reference ranges
  • Requires careful timing and sample handling

Overview

VWF testing is a complex panel of laboratory assessments designed to evaluate both quantitative and qualitative aspects of von Willebrand Factor, crucial for proper hemostasis and platelet function.

Core VWF Assays

  1. VWF Antigen (VWF:Ag)
    • Measures total VWF protein quantity
    • Normal range: 50-200 IU/dL
    • Basic screening test for VWD
  2. VWF Activity Assays
    • VWF:RCo (Ristocetin Cofactor Activity)
      • Measures functional ability to bind platelets
      • Gold standard for VWF function
      • Normal range: 50-200 IU/dL
    • VWF:GPIbR (Gain-of-function GPIb binding assay)
      • Alternative to VWF:RCo
      • More precise and reproducible
      • Less affected by technical variables
  3. VWF:CB (Collagen Binding)
    • Assesses binding to subendothelial collagen
    • Important for high molecular weight multimer function
    • Helps differentiate VWD subtypes

Additional Specialized Tests

  • VWF Multimer Analysis
    • Evaluates size distribution of VWF molecules
    • Critical for typing VWD variants
    • Complex technical requirements
  • FVIII:C (Factor VIII Activity)
    • Often included in VWF assessment
    • Reflects VWF's carrier function
    • Important for phenotype assessment

Diagnostic Applications

  • Primary VWD Diagnosis
    • Initial screening
    • Type differentiation
    • Severity assessment
  • Monitoring
    • Treatment response
    • Prophylaxis adequacy
    • Perioperative management
  • Risk Assessment
    • Preoperative evaluation
    • Bleeding risk stratification
    • Family screening

Specific Clinical Scenarios

  • Unexplained bruising or bleeding
  • Family history of bleeding disorders
  • Preoperative assessment
  • Heavy menstrual bleeding in adolescents
  • Excessive bleeding after minor procedures

Age-Specific Considerations

  • Neonates and Infants
    • Higher baseline levels
    • Age-specific reference ranges crucial
    • Developmental hemostasis impact
  • Children and Adolescents
    • Gradual normalization to adult ranges
    • Puberty-related changes
    • Activity level considerations

Result Patterns and Significance

  • Type 1 VWD Pattern
    • Proportional reduction in VWF:Ag and activities
    • Normal multimer distribution
    • VWF:RCo/VWF:Ag ratio > 0.7
  • Type 2 VWD Patterns
    • Discordant activity and antigen levels
    • Abnormal multimer patterns
    • Variant-specific characteristics
  • Type 3 VWD Pattern
    • Virtually absent VWF
    • Very low FVIII
    • No detectable multimers

Preanalytical Variables

  • Sample Collection
    • Proper citrate concentration
    • Fill volume requirements
    • Temperature control
    • Processing time limits
  • Patient Factors
    • Recent exercise
    • Stress levels
    • Time of day
    • Medications

Quality Assurance

  • Laboratory Standards
    • Method validation
    • Internal quality control
    • External quality assessment
  • Result Verification
    • Repeat testing protocols
    • Critical value handling
    • Result correlation
Further Reading


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