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Thrombotic thrombocytopenic purpura

Last reviewed: 25 Jun 2024
Last updated: 16 Mar 2022

Summary

Definition

History and exam

Key diagnostic factors

  • nonspecific prodrome
  • severe neurologic symptoms (coma, focal abnormalities, seizures)
  • mild neurologic symptoms (headache, confusion)
  • fever
Full details

Other diagnostic factors

  • age 30 to 50 years
  • digestive symptoms (nausea, vomiting, diarrhea, abdominal pain)
  • weakness
  • bleeding symptoms (purpura, ecchymosis, menorrhagia)
Full details

Risk factors

  • black ethnicity
  • female gender
  • obesity
  • pregnancy (near term or postpartum period)
  • cancer therapies
  • HIV infection
  • bone marrow transplantation
  • antiplatelet agents
  • quinine
Full details

Diagnostic tests

1st tests to order

  • platelet count
  • hemoglobin
  • haptoglobin
  • peripheral smear
  • reticulocyte count
  • urinalysis
  • BUN and creatinine
  • direct Coombs test
Full details

Tests to consider

  • ADAMTS-13 activity assay and inhibitor titers
Full details

Treatment algorithm

ACUTE

acquired (idiopathic) TTP: acute episode

ONGOING

acquired (idiopathic) TTP: following resolution of acute episode

Contributors

Authors

Sandeep K. Rajan, MD

Associate Professor of Medicine

Division of Hematology and Medical Oncology

Vanderbilt University

Nashville

Tennessee

Disclosures

SKR has received honoraria for advisory consultancy and speakers bureau, and received research funds from Alexion, Novo-Nordisk, Sanofi and Appelis.

Acknowledgements

Dr Sandeep K. Rajan would like to gratefully acknowledge Dr Rebecca Fischer Connor, a previous contributor to this topic.

Disclosures

RFC declares that she has no competing interests.

Peer reviewers

James N. George, MD

George Lynn Cross Professor

Department of Biostatistics and Epidemiology

University of Oklahoma Health Sciences Center

Oklahoma City

OK

Disclosures

JNG declares that he has no competing interests.

Christoph Pechlaner, MD

Associate Professor of Medicine

Department of Internal Medicine

Innsbruck Medical University

Innsbruck

Austria

Disclosures

CP declares that he has no competing interests.

  • Thrombotic thrombocytopenic purpura images
  • Differentials

    • Hemolytic uremic syndrome (HUS)
    • Atypical hemolytic uremic syndrome (aHUS)
    • Hypertension, malignant
    More Differentials
  • Guidelines

    • ISTH guidelines for the diagnosis of thrombotic thrombocytopenic purpura
    • Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies
    More Guidelines
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