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Background and Presentation of Thrombotic Thrombocytopenic Purpura

  • Clay Cohen
Chapter

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy (TMA) characterized by platelet-rich microthrombi which occlude capillaries and result in thrombocytopenia, hemolytic anemia, and organ damage. The mortality rate is about 90% in untreated individuals, carrying significant long-term morbidity for survivors. TTP is a rare condition, having many clinical features that overlap other TMA syndromes and more common hematology disorders. Timely recognition and treatment are critical for patient survival. The “classic pentad” of TTP (thrombocytopenia, microangiopathic hemolytic anemia, neurologic abnormalities, renal failure, and fever) is an uncommon presentation as TTP frequently presents with vague and nonspecific clinical signs and symptoms. The cause is a marked decrease in the von Willebrand factor-cleaving protease, ADAMTS-13, either through inhibitory antibodies or a congenital deficiency of the enzyme.

Keywords

Thrombotic thrombocytopenic purpura Thrombotic microangiopathy ADAMTS13 Microangiopathic hemolytic anemia Thrombocytopenia Upshaw-Schulman syndrome 

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Pediatric Hematology and OncologyBaylor College of MedicineHoustonUSA

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