Acquired thrombotic thrombocytopenic purpura

Acquired thrombotic thrombocytopenic purpura is a rare, life-threatening autoimmune thrombotic microangiopathy primarily affecting adults Clinically, it is characterized by severe consumptive thrombocytopenia, microangiopathic hemolytic anemia with schistocytes, and systemic tissue ischemia that most frequently compromises the central nervous system, heart, and kidneys

Acquired thrombotic thrombocytopenic purpura is a rare, life-threatening autoimmune thrombotic microangiopathy primarily affecting adults

The condition is driven by autoantibodies that inhibit or clear the von Willebrand factor-cleaving protease ADAMTS13, leading to an accumulation of ultra-large von Willebrand factor multimers

The condition is driven by autoantibodies that inhibit or clear the von Willebrand factor-cleaving protease ADAMTS13, leading to an accumulation of ultra-large von Willebrand factor multimers Clinically, it is characterized by severe consumptive thrombocytopenia, microangiopathic hemolytic anemia with schistocytes, and systemic tissue ischemia that most frequently compromises the central nervous system, heart, and kidneys

This deficiency triggers widespread, spontaneous microvascular platelet aggregation and microthrombi formation throughout the vascular bed Clinically, it is characterized by severe consumptive thrombocytopenia, microangiopathic hemolytic anemia with schistocytes, and systemic tissue ischemia that most frequently compromises the central nervous system, heart, and kidneys

This deficiency triggers widespread, spontaneous microvascular platelet aggregation and microthrombi formation throughout the vascular bed