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The enzyme ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats) cleaves large multimers of von Willebrand factor (vWF), which decreases their activity. Congenital or acquired deficiency of ADAMTS13 is associated with an increase in ultra-large multimers of vWF, formation of platelet aggregates, thrombocytopenia, and vascular occlusion, resulting in microangiopathic hemolytic anemia. Some patients with idiopathic thrombotic microangiopathy (TMA) have autoantibodies against ADAMTS13, but the relation among vWF, ADAMTS13 activity, and ADAMTS13 autoantibodies has been unclear.
In a retrospective study review of data from 35 consecutive French patients with first episodes of TMA and no history of sepsis, cancer, HIV inf…