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Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome in Cancer Patients

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Oncologic Critical Care

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Abstract

Two thrombotic microangiopathies that may be managed in an intensive care unit setting include thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Although these two disorders may have similar characteristics, the underlying pathophysiology, workup, management, and prognosis are very different. Clinicians should consider these disorders when patients are admitted with microangiopathic hemolytic anemia and thrombocytopenia.

Thrombotic thrombocytopenic purpura, most commonly arises from development of an autoantibody to the metalloproteinase ADAMTS13 enzyme, is considered a medical emergency and warrants emergent initiation of treatment, typically with therapeutic plasma exchange, even prior to confirmation of the diagnosis. Hemolytic uremic syndrome is now separated into two distinct categories: primary versus secondary. Primary HUS, formerly diarrhea-negative or “atypical” HUS, is now referred to as complement-mediated HUS and is caused by a complement factor autoantibody or either loss- or gain-of-function mutations that dysregulate self-preservation against complement-induced membrane attack complex. Secondary HUS, formerly referred to as diarrhea-positive HUS, is caused by shiga toxin-induced damage to endothelial cells, renal cells, and epithelial cells resulting in a proinflammatory and prothrombotic state.

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Authors and Affiliations

  1. Department of Internal Medicine – Section on Pulmonary, Critical Care, Allergy and Immunologic Diseases, Section on Hematology and Oncology, Department of Anesthesiology – Section on Critical Care Medicine, Wake Forest University School of Medicine, Winston-Salem, NC, USA

    Peter J. Miller

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  1. Department of Critical Care, University of Texas MD Anderson Cancer C Department of Critical Care, Houston, TX, USA

    Joseph L. Nates

  2. Department of Critical Care, University of Texas MD Anderson Cancer C Department of Critical Care, Houston, TX, USA

    Kristen J. Price

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Miller, P.J. (2019). Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome in Cancer Patients. In: Nates, J., Price, K. (eds) Oncologic Critical Care. Springer, Cham. https://doi.org/10.1007/978-3-319-74698-2_91-1

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  • DOI: https://doi.org/10.1007/978-3-319-74698-2_91-1

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