L. Fianchi
Abstract
Thrombotic microangiopathies (TMAs) are rare and life threatening diseases. In recent years, there have been major advances in understanding the pathophysiology, classification, and treatment of these disorders, including the introduction of new drugs. Early differential diagnosis is crucial for prompt treatment to reduce high mortality rates and late organ damage.
The classic forms of thrombotic microangiopathy are thrombotic thrombocytopenic purpura (TTP), typical hemolytic uremic syndrome (HUS) associated with toxin producing bacteria, atypical HUS (aHUS) due to dysregulation of alternative complement C3 convertase attributable to genetic causes, and HUS secondary to coexisting disease...
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