Thrombocytopenia in hospitalized patients: approach to the patient with thrombotic microangiopathy

摘要

Thrombotic microangiopathies (TMAs), specifically, thrombotic thrombocytopenic purpura (TTP) and complement-mediated hemolytic uremic syndrome (CM-HUS) are acute life-threatening disorders that require prompt consideration, diagnosis, and treatment to improve the high inherent mortality and morbidity. Presentation is with microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) and variable organ symptoms resulting from microvascular thrombi. Neurological and cardiac involvement is most common in TTP and associated with poorer prognosis and primarily renal involvement in CM-HUS. TTP is confirmed by severe ADAMTS13 deficiency (which can be undertaken in real time) and CM-HUS by an abnormality in complement regulators, confirmed by mutational analysis (in 60% to 70% of cases) or the presence of Factor H antibodies (which may not be available for weeks or months). Plasma exchange (PEX) should be started as soon as possible following consideration of these TMAs. Differentiation of the diagnosis requires specific treatment pathways thereafter (immunosuppression primarily for TTP and complement inhibitor therapy for CM-HUS). As the diagnosis is based on MAHAT, there are a number of other medical situations that need to be excluded and these are discussed within the article. Other differentials presenting as TMAs may also be associated with micro- or macrovascular thrombosis, yet are more likely to be due to direct endothelial damage, many of which do not have a clear therapeutic benefit with PEX.