Since the SCA patients have elevated ECHb in plasma, as estimated up to 410 μg/mL during their vasoocclusive crisis [21–23], we speculated that excessive ECHb could impair VWF cleavage by ADAMTS13 in vivo and promote accumulation of hyperactive VWF multimers in patients' plasma. Here, VWF is linked to autosomal dominant cerebellar ataxia.