| Type | Clinical features | Laboratory findings | Comments on treatment |
| Type 1 (partial quantitative deficiency) | - Accounts for approximately 75% of individuals with VWD
- Variable bleeding severity from mild to severe
- AD inheritance
| - VWF activity and antigen decreased concordantly
- Factor VIII activity normal or reduced
- RIPA decreased (may be normal in mild disease)
- Multimer electrophoresis: All multimers present and uniformly decreased
- In type 1C (increased clearance), the VWF level at 4 hours post DDAVP trial shows rapid reduction in VWF
| - DDAVP* in most patients
- VWF concentrates in moderate, severe, and type 1C patients
|
| Type 2 (qualitative variant) |
| Type 2A (selective deficiency of HMW multimers, reduced binding to platelet GPIb) | - Accounts for approximately 10 to 20% of individuals with VWD
- Moderate to severe bleeding
- Mostly AD; occasional AR inheritance
| - VWF activity decreased out of proportion to VWF antigen
- Factor VIII activity normal or reduced
- RIPA decreased
- Multimer electrophoresis: Large multimers decreased
| - DDAVP*
- VWF concentrates in moderate and severe patients
- Follow VWF levels
|
| Type 2B (enhanced binding of HMW VWF multimers to platelet GPIb; may have decrease in circulating HMW multimers) | - Accounts for approximately 5% of individuals with VWD
- Moderate to severe bleeding
- Thrombocytopenia
- AD inheritance
| - VWF activity decreased out of proportion to VWF antigen
- Factor VIII activity normal or reduced
- Thrombocytopenia
- RIPA increased
- Multimer electrophoresis: Usually decreased large multimers
| - DDAVP* should be used with caution; it may be used to treat minor bleeding if a trial of DDAVP performed when the patient is not bleeding has demonstrated that the platelet count drop is temporary
- VWF concentrates in moderate and severe patients
|
| Type 2M (reduced binding of VWF to platelet GPIb) | - Uncommon
- Moderate to severe bleeding
- AD or AR inheritance
| - VWF activity decreased out of proportion to VWF antigen
- Factor VIII activity normal or decreased
- RIPA decreased
- Multimer electrophoresis: All multimers present and uniformly decreased
| - DDAVP*
- VWF concentrates in moderate and severe patients
|
| Type 2N (reduced binding of VWF to factor VIII) | - Uncommon
- Clinically similar to hemophilia A with joint, soft tissue, and urinary bleeding
- AR inheritance
| - VWF activity and antigen normal
- Factor VIII levels low (5 to 15%)
- RIPA normal
- Multimer electrophoresis: Normal
| - DDAVP*
- VWF concentrates
- Monitor VWF and factor VIII levels
|
| Type 3 (severe quantitative deficiency/absent VWF) | - Rare
- Clinically similar to hemophilia A with joint and soft tissue bleeding in addition to mucocutaneous bleeding
- AR inheritance
| - VWF activity and antigen absent or markedly decreased
- Factor VIII levels low (1 to 10%)
- RIPA absent or very low
- Multimer electrophoresis: Undetectable or too faint to visualize
| - VWF concentrates
- Factor VIII replacement
- Do not use DDAVP to treat bleeding (will not be effective)
|
