Figure 2.

Type 2 VWD Variants. Panel A: Type 2A VWD has variants in the A2 domain, causing decreased platelet binding ability and loss of HMWM, and can also have variants in the N and C terminal multimerization domains. Panel B: Type 2B VWD has variants in the A1 domain which increase platelet (PLT) binding to VWF and result in clearance of the VWF:platelet complex. Panel C: Type 2M VWD has variants in the A1 and A3 domains, which cause decreased platelet (PLT) binding and may or may not cause decreased collagen (COL) binding. Panel D: Type 2N VWD has variants in the D′ and D3 domains which cause decreased factor VIII (FVIII) binding to VWF and subsequent FVIII clearance from circulation. HMWM, high molecular weight multimers; VWD, von Willebrand disease