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Diagnosis and Management of Thrombotic Thrombocytopenic Purpura

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Evidence-Based Critical Care

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Abstract

Thrombotic thrombocytopenic purpura (TTP) is a specific type of primary thrombotic microangiopathy syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ failure. Although relatively rare, if left unrecognized and untreated, multisystem organ failure ensues and survival is poor. Early use of therapeutic plasma exchange has significantly improved mortality and the subsequent risk of relapse. Therefore, prompt recognition of this medical emergency is essential. Diagnosis rests on maintaining a low index of suspicion for TTP in a patient presenting with microangiopathic hemolytic anemia and thrombocytopenia in the absence of an alternative diagnosis. Adjunctive testing including ADAMTS13 activity levels and ADAMTS13 autoantibody testing may help confirm the diagnosis and predict relapse risk. In addition to plasma exchange, aggressive immunosuppression with high-dose corticosteroids is recommended. Refractory TTP may require intensification of plasma exchange and the addition of rituximab. In this chapter, we will review the essential practical diagnostic and management considerations for TTP in the context of a clinical case.

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Authors and Affiliations

  1. Texas Intensivist, Pulmonary, and Sleep Medicine, San Antonio, TX, USA

    Bravein Amalakuhan

  2. Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of Texas Health San Antonio, and the South Texas Veterans Health Care System, San Antonio, TX, USA

    Anoop M. Nambiar

Authors
  1. Bravein Amalakuhan
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  2. Anoop M. Nambiar
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Correspondence to Anoop M. Nambiar .

Editor information

Editors and Affiliations

  1. Division of Pulmonary and Critical Care, University of Michigan, Ann Arbor, MI, USA

    Robert C. Hyzy

  2. Division of Pulmonary and Critical Care, University of Michigan, Ann Arbor, MI, USA

    Jakob McSparron

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Amalakuhan, B., Nambiar, A.M. (2020). Diagnosis and Management of Thrombotic Thrombocytopenic Purpura. In: Hyzy, R.C., McSparron, J. (eds) Evidence-Based Critical Care. Springer, Cham. https://doi.org/10.1007/978-3-030-26710-0_76

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  • DOI: https://doi.org/10.1007/978-3-030-26710-0_76

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-26709-4

  • Online ISBN: 978-3-030-26710-0

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