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Trombocitemia essencial

Last reviewed: 4 Aug 2025
Last updated: 03 Dec 2024

Summary

Definition

History and exam

Key diagnostic factors

  • eritromelalgia
  • esplenomegalia
  • trombose arterial e venosa
  • sangramento
  • livedo reticular
Full details

Other diagnostic factors

  • idade ≥60 anos
  • sexo feminino
  • cefaleia
  • tontura, dor torácica, vertigem e parestesia
  • síncope e convulsões
  • distúrbios visuais transitórios
  • priapismo
Full details

Risk factors

  • mutações genéticas (JAK2 V617F, CALR ou MPL)
  • etnia negra
  • idade ≥60 anos
  • sexo feminino
Full details

Diagnostic investigations

1st investigations to order

  • Hemograma completo com diferencial
  • esfregaço de sangue periférico
  • perfis séricos do ferro
Full details

Investigations to consider

  • proteína C-reativa
  • velocidade de hemossedimentação (VHS)
  • fibrinogênio
  • biópsia e histopatologia da medula óssea
  • teste de mutação genética (JAK2 V617F, CALR e MPL)
  • testes citogenéticos e moleculares: BCR::ABL1
Full details

Treatment algorithm

ACUTE

trombose com risco de vida, complicações neurológicas graves relacionadas à trombocitose ou sangramento grave

ONGOING

não gestante: risco muito baixo, baixo ou intermediário

não gestante: alto risco

gestante: risco muito baixo, baixo ou intermediário

gestante: alto risco

Contributors

Authors

Debabrata Mukherjee, MD, FACC
Debabrata Mukherjee

Chairman, Department of Internal Medicine

Professor of Internal Medicine

Texas Tech University Health Sciences Center at El Paso

El Paso

TX

Disclosures

DM declares that he has no competing interests.

Peer reviewers

Tony Kan, MD

Senior Staff Physician

Henry Ford Medical Center

Sterling Heights

MI

Disclosures

TK declares that he has no competing interests.

Uri Rozovski, MD

Department of Cardiology

Tel Aviv Sourasky Medical Center

Tel Aviv

Israel

Disclosures

UR declares that he has no competing interests.

David A. Garcia, MD

Professor

Division of Hematology

University of Washington Seattle

WA

Disclosures

DAG declares that he has no competing interests.

Bethany Samuelson, MD

Fellow

Division of Hematology

University of Washington

Seattle

WA

Disclosures

BS declares that she 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.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: myeloproliferative neoplasms [internet publication].Full text

Tefferi A, Vannucchi AM, Barbui T. Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management. Am J Hematol. 2024 Apr;99(4):697-718.Full text  Abstract

Harrison CN, Butt N, Campbell P, et al. Modification of British Committee for Standards in Haematology diagnostic criteria for essential thrombocythaemia. Br J Haematol. 2014 Nov;167(3):421-3. Abstract

Vannucchi AM, Barbui T, Cervantes F, et al. Philadelphia chromosome-negative chronic myeloproliferative neoplasms: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2015 Sep;26 Suppl 5:v85-99.Full text  Abstract

Harrison CN, Bareford D, Butt N, et al; British Committee for Standards in Haematology. Guideline for investigation and management of adults and children presenting with a thrombocytosis. Br J Haematol. 2010 May;149(3):352-75.Full text  Abstract

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
  • Trombocitemia essencial images
  • Differentials

    • Trombocitose (reativa) secundária
    • Trombocitose espúria
    • Trombocitemia essencial familiar
    More Differentials
  • Guidelines

    • NCCN clinical practice guidelines in oncology: myeloproliferative neoplasms
    • The 5th edition of the World Health Organization (WHO) classification of haematolymphoid tumours: myeloid and histiocytic/dendritic neoplasms
    More Guidelines
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