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Acquired TTP: Initial treatment

Author
James N George, MD
Section Editor
Lawrence LK Leung, MD
Deputy Editor
Jennifer S Tirnauer, MD

INTRODUCTION

Classification of thrombotic thrombocytopenic purpura (TTP) and related syndromes such as hemolytic uremic syndrome (HUS) is evolving. We now use TTP to refer to the thrombotic microangiopathy (TMA) caused by severely reduced activity of the von Willebrand factor-cleaving protease ADAMTS13. We divide TTP into acquired and hereditary syndromes, due to an autoantibody against ADAMTS13 and ADAMTS13 gene mutations, respectively. TTP is characterized by small-vessel platelet-rich thrombi, thrombocytopenia, and microangiopathic hemolytic anemia. In addition, some patients may have neurologic abnormalities, mild renal insufficiency, and low-grade fever.

An overview of our approach to the classification of TTP and related syndromes is presented separately. (See "Approach to the patient with suspected TTP, HUS, or other thrombotic microangiopathy (TMA)".)

Acquired autoimmune 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. This topic reviews our approach to the initial therapy for children and adults with acquired autoimmune TTP, defined by severe ADAMTS13 deficiency (ie, activity level <10 percent) caused by an inhibitory autoantibody.

The following aspects of care for individuals with acquired, autoimmune TTP are discussed in detail separately:

Clinical manifestations and diagnosis – (See "Diagnosis of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome in adults".)

                                 

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Literature review current through: Jul 2015. | This topic last updated: Jun 26, 2015.
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