Deficiency of this protease results in unusually large VWF (UL-VWF) binding spontaneously with platelets, leading to microvascular thrombosis, microangiopathic hemolysis, and subsequent ischemia, hypoxia, and dysfunction of the affected organs.[2] The clinical presentation of this patient included severe thrombocytopenia with anemia, central nervous system injury, and recurrence of nephropathy. Here, VWF is linked to anemia (phenotype).