Raife et al., Curr Opin Hematol 2000
(Purpura, Thrombotic Thrombocytopenic...) :
The discovery of vWF cleaving protease activity in normal plasma, and its deficiency in thrombotic thrombocytopenic purpura (TTP) patients, provides additional support for a pathologic role of ultra-large vWF in TTP Veyradier et al., Blood 2001
(Hemolytic-Uremic Syndrome...) :
Retrospective studies of patients with thrombotic microangiopathies ( TMAs ) have shown that a deficient activity of von Willebrand factor (vWF) cleaving protease is involved in thrombotic thrombocytopenic purpura (TTP) but not in the hemolytic-uremic syndrome ( HUS )
Niemann et al., Ugeskr Laeger 2009
(Pregnancy Complications, Cardiovascular...) :
TTP is due to reduced activity of the von Willebrand factor which cleaves ADAMTS13