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Thrombotic thrombocytopenic purpura

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



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


acquired (idiopathic) TTP: acute episode


acquired (idiopathic) TTP: following resolution of acute episode



Sandeep K. Rajan, MD

Associate Professor of Medicine

Division of Hematology and Medical Oncology

Vanderbilt University




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


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


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



JNG declares that he has no competing interests.

Christoph Pechlaner, MD

Associate Professor of Medicine

Department of Internal Medicine

Innsbruck Medical University




CP declares that he has no competing interests.

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  • Differentials

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