What is the classic pentad in thrombotic thrombocytopenic purpura (TTP)?
Thrombocytopenia Microangiopathic hemolytic anemia (MAHA) Neurological changes (often fluctuating) Renal impairment Fever
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What is the cause of acquired (also called immune) thrombotic thrombocytopenic purpura (TTP)?
Autoantibodies to ADAMTS13
What is thrombotic thrombocytopenic purpura (TTP)?
A thrombotic microangiopathy (TMA) caused by deficiency of ADAMTS13, a von Willebrand factor cleaving protease, which leads to platelet-mediated occlusion of microvessels, thrombocytopenia, microangiopathic hemolytic anemia,…
What is the pathogenesis of thrombotic thrombocytopenic purpura (TTP)?
ADAMTS13 deficiency leads to reduced cleavage of ultra large vWF multimers, which leads to increased platelet aggregation and occlusion of small blood vessels.
What is another name for congenital thrombotic thrombocytopenic purpura (TTP)?
Upshaw-Schulman syndrome
What is the cause of thrombotic thrombocytopenic purpura (TTP)?
Congenital or acquired deficiency of the von Willebrand factor (vWF) cleaving protein, ADAMTS13
What is the most important treatment modality in thrombotic thrombocytopenia purpura (TTP)?
Plasma exchange