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Acquired TTP: Clinical manifestations and diagnosis

James N George, MD
Adam Cuker, MD, MS
Section Editor
Lawrence LK Leung, MD
Deputy Editor
Jennifer S Tirnauer, MD


Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy caused by severely reduced activity of the von Willebrand factor-cleaving protease ADAMTS13. It is characterized by small-vessel platelet-rich thrombi that cause thrombocytopenia, microangiopathic hemolytic anemia, and sometimes organ damage. TTP is a medical emergency that is almost always fatal if appropriate treatment is not initiated promptly. With appropriate treatment, survival rates of up to 90 percent are possible.

TTP can be acquired, due to an autoantibody inhibitor, or hereditary, due to inherited mutations in ADAMTS13. This topic reviews the clinical manifestations and our approach to the diagnosis of acquired autoimmune TTP. Our approach to evaluating patients with other suspected thrombotic microangiopathies (including hereditary TTP) is presented in separate topic reviews:

Suspected TTP or hemolytic uremic syndrome (HUS), diagnosis unclear – (see "Approach to the patient with suspected TTP, HUS, or other thrombotic microangiopathy (TMA)")

Suspected hereditary TTP – (see "Hereditary thrombotic thrombocytopenic purpura (TTP)")

Suspected drug-induced thrombotic microangiopathy (also called drug-induced TTP) – (see "Drug-induced thrombotic microangiopathy")

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Literature review current through: Oct 2017. | This topic last updated: Nov 07, 2017.
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