摘要
Thrombotic microangiopathy (TMA) is a group of diseases that are life-threatening and can lead to end organ damage (EOD) due to ischemia caused by microthrombi in capillaries and arterioles. TMAs can affect any organ system but usually affect the kidney, intestines, and nervous system. The triad of TMA is Coombs-negative hemolytic anemia with schistocytes seen on peripheral smear, thrombocytopenia (platelets under 150,000 or a decrease of 25% or more from baseline), and evidence of ischemic EOD. Primary TMAs include Thrombotic Thrombocytopenic Purpura (TTP), Hemolytic Uremic Syndrome (HUS) and atypical HUS (aHUS). Pathophysiologically, all of these diseases are caused by aggregation of von Willebrand Factor (vWF) multimers, via different mechanisms, which eventually leads to thrombus formation. TTP and aHUS benefit from plasma exchange (PEX), whereas HUS is treated symptomatically. Urgent recognition with timely treatment is crucial to managing these potentially life-threatening conditions.
Thrombotic microangiopathy (TMA) is a group of diseases that are life-threatening and can lead to end organ damage (EOD) due to ischemia caused by microthrombi in capillaries and arterioles. TMAs can affect any organ system but usually affect the kidney, intestines, and nervous system. The triad of TMA is Coombs-negative hemolytic anemia with schistocytes seen on peripheral smear, thrombocytopenia (platelets under 150,000 or a decrease of 25% or more from baseline), and evidence of ischemic EOD. Primary TMAs include Thrombotic Thrombocytopenic Purpura (TTP), Hemolytic Uremic Syndrome (HUS) and atypical HUS (aHUS). Pathophysiologically, all of these diseases are caused by aggregation of von Willebrand Factor (vWF) multimers, via different mechanisms, which eventually leads to thrombus formation. TTP and aHUS benefit from plasma exchange (PEX), whereas HUS is treated symptomatically. Urgent recognition with timely treatment is crucial to managing these potentially life-threatening conditions.
