Table 3. Synoptic representation of the differences and similarities between type 2B VWD patients with or without large VWF multimers.
2B VWD patients | Platelet count | *SPA | °MADR | VWF:Ag |
§VWF:RCo ratio |
^VWF:CB ratio |
Platelet VWF:Ag |
Large multimers |
VWF activation state |
VWF mutation in A1 domain |
Bleeding tendency | Therapy | |||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Pre | Post | ||||||||||||||
DDAVP | DDAVP | Platelet | VWF/FVIII concentrates | ||||||||||||
Without multimers | ↓/N | ↓↓ | Present | ↓ | ↓ | ↓↓↓ | ↓↓↓ | N | Absent | ↑ | Present | ↑↑ | No/Yes | No | Yes |
With multimers | N | N | Present | ↓ | N/↓ | N | N | N/↓ | Present | ↑ | Present | ↑ | Yes | Yes | Yes |
*Spontaneous platelet aggregation
°Minimal aggregating dose of ristocetin
§VWF:RCo/VWF:Ag
^VWF:CB/VWF:Ag.
N = normal; ↓ = reduced; ↑ = increased.