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Background and Presentation of Thrombotic Thrombocytopenic Purpura

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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.

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  1. Department of Pediatric Hematology and Oncology, Baylor College of Medicine, Houston, TX, USA

    Clay Cohen MD

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    Jenny M. Despotovic

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Cohen, C. (2018). Background and Presentation of Thrombotic Thrombocytopenic Purpura. In: Despotovic, J. (eds) Immune Hematology. Springer, Cham. https://doi.org/10.1007/978-3-319-73269-5_8

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