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Thrombotic thrombocytopenic purpura

Última revisão: 9 Aug 2025
Última atualização: 16 Mar 2022

Resumo

Definição

História e exame físico

Principais fatores diagnósticos

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

Outros fatores diagnósticos

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

Fatores de risco

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

Investigações diagnósticas

Primeiras investigações a serem solicitadas

  • platelet count
  • hemoglobin
  • haptoglobin
  • peripheral smear
  • reticulocyte count
  • urinalysis
  • BUN and creatinine
  • direct Coombs test
Detalhes completos

Investigações a serem consideradas

  • ADAMTS-13 activity assay and inhibitor titers
Detalhes completos

Algoritmo de tratamento

AGUDA

acquired (idiopathic) TTP: acute episode

CONTÍNUA

acquired (idiopathic) TTP: following resolution of acute episode

Colaboradores

Autores

Sandeep K. Rajan, MD

Associate Professor of Medicine

Division of Hematology and Medical Oncology

Vanderbilt University

Nashville

Tennessee

Declarações

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

Agradecimentos

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

Declarações

RFC declares that she has no competing interests.

Revisores

James N. George, MD

George Lynn Cross Professor

Department of Biostatistics and Epidemiology

University of Oklahoma Health Sciences Center

Oklahoma City

OK

Declarações

JNG declares that he has no competing interests.

Christoph Pechlaner, MD

Associate Professor of Medicine

Department of Internal Medicine

Innsbruck Medical University

Innsbruck

Austria

Declarações

CP declares that he has no competing interests.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

Referências

Nossas equipes internas de editoria e de evidências trabalham em conjunto com colaboradores internacionais especializados e pares revisores para garantir que forneçamos acesso às informações o mais clinicamente relevantes possível.

Principais artigos

Scully M, Cataland S, Coppo P, et al. Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies. J Thromb Haemost. 2017 Feb;15(2):312-22.Texto completo  Resumo

Scully M, Hunt BJ, Benjamin S, et al. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012 Aug;158(3):323-35.Texto completo  Resumo

Terrell DR, Williams LA, Vesely SK, et al. The incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency. J Thromb Haemost. 2005 Jul;3(7):1432-6.Texto completo  Resumo

Moschowitz E. An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries. Arch Intern Med. 1925;36:89.

Moore JC, Hayward CP, Warkentin TE, et al. Decreased von Willebrand factor protease activity associated with thrombocytopenic disorders. Blood. 2001;98:1842-1846.Texto completo  Resumo

Banno F, Kokame K, Okuda T, et al. Complete deficiency in ADAMTS13 is prothrombotic, but it alone is not sufficient to cause thrombotic thrombocytopenic purpura. Blood. 2006 Apr 15;107(8):3161-6.Texto completo  Resumo

Veyradier A, Meyer D. Thrombotic thrombocytopenic purpura and its diagnosis. J Thromb Haemost. 2005 Nov;3(11):2420-7. Resumo

Rock GA, Shumak KH, Buskard NA, et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med. 1991 Aug 8;325(6):393-7. Resumo

Cuker A, Cataland SR, Coppo P, et al. Redefining outcomes in immune TTP: an international working group consensus report. Blood. 2021 Apr 8;137(14):1855-61.Texto completo  Resumo

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.
  • Thrombotic thrombocytopenic purpura images
  • Diagnósticos diferenciais

    • Hemolytic uremic syndrome (HUS)
    • Atypical hemolytic uremic syndrome (aHUS)
    • Hypertension, malignant
    Mais Diagnósticos diferenciais
  • Diretrizes

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