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Treatment and prognosis of thrombotic thrombocytopenic purpura-hemolytic uremic syndromes in adults

INTRODUCTION

The treatment of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) in the adult will be discussed here [1,2].

Discussions of the causes and diagnosis of TTP-HUS in adults, and of the treatment of HUS in children, are presented separately. (See "Causes of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome in adults" and "Diagnosis of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome in adults" and "Treatment and prognosis of Shiga toxin associated (typical) hemolytic uremic syndrome in children".)

DISEASE OVERVIEW

Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are systemic disorders characterized by endothelial injury and the formation of small vessel platelet rich thrombi. The resulting thrombotic microangiopathy produces microangiopathic hemolytic anemia and thrombocytopenia; presenting features may also include neurologic and/or renal abnormalities.

Although some studies appear to distinguish between TTP and HUS in adults, the presenting features are essentially the same in most patients [3-6].

  • In a few patients, neurologic abnormalities are dominant and renal failure is minimal or not present; these patients are considered by some to represent TTP.
  • When renal failure is dominant and neurologic abnormalities are minimal or absent, the disorder is considered by some to represent HUS.
  • Some patients present with severe neurologic abnormalities (eg, focal neurologic abnormalities, seizures, coma) together with renal failure.
  • Many patients have neither neurologic nor renal function abnormalities. For example, approximately one-half of our patients with TTP-HUS associated with a severe (ie, <10 percent) deficiency of ADAMTS13 activity had no or minimal neurologic abnormalities and no evidence for renal failure.

                                                       

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Literature review current through: Apr 2013. | This topic last updated: Mar 12, 2013.
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