Thrombotic thrombocytopenic purpura

Last reviewed: 28 Apr 2022
Last updated: 16 Mar 2022

Summary

Definition

History and exam

Key diagnostic factors

  • non-specific prodrome
  • severe neurological symptoms (coma, focal abnormalities, seizures)
  • mild neurological symptoms (headache, confusion)
  • fever
More key diagnostic factors

Other diagnostic factors

  • age 30 to 50 years
  • digestive symptoms (nausea, vomiting, diarrhoea, abdominal pain)
  • weakness
  • bleeding symptoms (purpura, ecchymosis, menorrhagia)
Other diagnostic factors

Risk factors

  • black ethnicity
  • female gender
  • obesity
  • pregnancy (near term or post-partum period)
  • cancer therapies
  • HIV infection
  • bone marrow transplantation
  • antiplatelet agents
  • quinine
More risk factors

Diagnostic investigations

1st investigations to order

  • platelet count
  • haemoglobin
  • haptoglobin
  • peripheral smear
  • reticulocyte count
  • urinalysis
  • urea and creatinine
  • direct Coombs' test
More 1st investigations to order

Investigations to consider

  • ADAMTS-13 activity assay and inhibitor titres
More investigations to consider

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

    • Haemolytic uraemic syndrome (HUS)
    • Atypical haemolytic uraemic 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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