Figure 1.
Supra large VWF multimers in 3 siblings (thrombotic thrombocytopenic purpura [TTP]) with hereditary ADAMTS13 deficiency compared to normal plasma (NP). Activation of ADAMTS13 in normal plasma (NP +a) results in complete proteolytic cleavage of VWF multimers, whereas VWF in the TTP patient (TTP +a) remains unchanged due to the lack of ADAMTS13.