TTP-HUS

TTP-HUS

The combination of thrombotic thrombocytopenic purpura–TTP and hemolytic-uremic syndrome–HUS, polar expressions of the same disease defined by a pentad of TTP features: Thrombocytopenia, microangiopathic hemolytic anemia, neurologic defects, fever, renal disease Clinical Abrupt onset in children after a viral URI; may be associated with a verotoxin in E coli-induced gastroenteritis; it may occur in pregnancy, at parturition, or during chemotherapy; spontaneously resolving renal failure–RF occurs in 60%–10% progress to chronic RF; RF is more common in E coli O157:H7 infections, which are linked to 16% of cases with HUS Lab Reticulocytosis, ↑ BR, ↑ FDPs without DIC, ↓ haptoglobin Management Most resolve spontaneously, others require high-dose corticosteroids Prognosis with prednisone and plasma exchange, 91% survival