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Púrpura trombocitopênica trombótica

  • Overview
  • Theory
  • Diagnosis
  • Management
  • Follow up
  • Resources
Last reviewed: 23 Sep 2025
Last updated: 16 Mar 2022

Summary

Definition

History and exam

Key diagnostic factors

  • pródromo inespecífico
  • sintomas neurológicos graves (coma, anormalidades focais, convulsões)
  • sintomas neurológicos leves (cefaleia, confusão)
  • febre
Full details

Other diagnostic factors

  • idade entre 30 e 50 anos
  • sintomas digestivos (náuseas, vômitos, diarreia, dor abdominal)
  • fraqueza
  • sintomas de sangramento (púrpura, equimoses, menorragia)
Full details

Risk factors

  • etnia negra
  • sexo feminino
  • obesidade
  • gestação (parto próximo ou período pós-parto)
  • terapias para câncer
  • Infecção pelo vírus da imunodeficiência humana (HIV)
  • transplante de medula óssea
  • agentes antiplaquetários
  • quinina
Full details

Diagnostic tests

1st tests to order

  • contagem plaquetária
  • hemoglobina
  • haptoglobina
  • esfregaço de sangue periférico
  • contagem de reticulócitos
  • urinálise
  • ureia e creatinina
  • teste de Coombs direto
Full details

Tests to consider

  • ensaios da atividade da enzima ADAMTS-13 e títulos dos inibidores
Full details

Treatment algorithm

ACUTE

PTT adquirida (idiopática): episódio agudo

ONGOING

PTT adquirida (idiopática): após a resolução de episódio agudo

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

利益声明

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.

参考文献

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关键文献

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.全文  摘要

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.全文  摘要

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.全文  摘要

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 Sep 15;98(6):1842-6.全文  摘要

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.全文  摘要

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

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. 摘要

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.全文  摘要

参考文献

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
  • Púrpura trombocitopênica trombótica images

  • 鉴别诊断

    • Síndrome hemolítico-urêmica (SHU)
    • Síndrome hemolítico-urêmica atípica (SHUa)
    • Hipertensão maligna
    更多 鉴别诊断

  • 指南

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