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||vWF-cleaving Protease Activity
||A severe decrease in ADAMTS-13 activity to less than 10% has been shown to be diagnostic for Thrombotic Thrombocytopenic Purpura (TTP), an illness characterized by thrombocytopenia, microangiopathic hemolytic anemia (MAHA), fever, renal dysfunction and central nervous system ischemia. TTP is often difficult to diagnose as well as differentiate from other thrombotic microangiopathies (TMA) such as hemolytic uremic syndrome (HUS) and atypical hemolytic uremic syndrome (aHUS).
TTP is characterized by the acquired or congenital deficiency of ADAMTS-13 activity. An antibody inhibitor will be present in roughly half of the cases diagnosed with idiopathic TTP.
Early diagnosis is paramount. Left untreated, TTP has a mortality rate above 90%; however, rapid diagnosis and treatment with plasma exchange improve the mortality rate to below 20%.
To maximize the clinical utility of this test, Machaon Diagnostics is offering ADAMTS-13 activity and inhibitor testing on a daily basis with clinical consultation.
Weekend testing is available to contracted clients. Please phone the lab for further information.