![]() Once thought to be interchangeable terms, TTP and HUS have distinctly different mechanisms. In recent years, our understanding of pathophysiology has improved considerably. ![]() 3 Regardless of the etiology, thrombotic microangiopathy is a hematologic emergency that requires prompt treatment. The incidence of TTP in adults is about 3 per 1 000 000, 2 and the incidence of HUS in children is about 3 per 100 000. Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are primary forms of thrombotic microangiopathies. It is a process of red blood cell destruction within the microvasculature accompanied by thrombocytopenia due to platelet activation and consumption. Microangiopathic hemolytic anemia is the hallmark of thrombotic microangiopathy. Primary care or emergency department physicians are often the first point of contact however, once the diagnosis is suspected, urgent referral is needed to a specialty service (e.g., hematology or nephrology) that can provide plasma exchange. Presenting symptoms may be nonspecific, but basic laboratory tests reveal a specific constellation of thrombocytopenia and anemia with red blood cell fragmentation on the blood film and evidence of hemolysis. 1 Though rare, thrombotic microangiopathies are life-threatening conditions that require urgent management. Thrombotic microangiopathies are a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia and microthrombi leading to ischemic tissue injury. ![]()
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