When viewing this topic in a different language, you may notice some differences in the way the content is structured, but it still reflects the latest evidence-based guidance.

Policitemia vera

  • Overview
  • Theory
  • Diagnosis
  • Management
  • Follow up
  • Resources
Last reviewed: 22 Sep 2025
Last updated: 03 Dec 2024

Summary

Definition

History and exam

Key diagnostic factors

  • presença de fatores de risco
  • características da trombose
Full details

Other diagnostic factors

  • características da hemorragia
  • cefaleia
  • fraqueza generalizada/fadiga
  • prurido
  • sudorese noturna e dor óssea
  • eritromelalgia
  • esplenomegalia
  • pletora/cianose avermelhada
  • zumbido
  • visão embaçada
  • artralgia
  • desconforto abdominal
  • hiperidrose (suor excessivo)
Full details

Risk factors

  • idade >60 anos
  • história de síndrome de Budd-Chiari (SBC)
  • membro da família afetado
  • Mutações na Janus quinase 2 (JAK2) (JAK2 V617F e exon 12 da JAK2)
Full details

Diagnostic investigations

1st investigations to order

  • hemoglobina
  • hematócritos
  • contagem leucocitária
  • contagem plaquetária
  • volume corpuscular médio (VCM)
  • esfregaço de sangue periférico
  • testes da função hepática
  • ferritina sérica
  • Teste de mutação do gene JAK2
  • biópsia da medula óssea
Full details

Investigations to consider

  • saturação do oxigênio
  • eritropoetina sérica
  • massa eritrocitária:
  • testes mutacionais adicionais
  • testes citogenéticos e moleculares: BCR::ABL1
  • ácido úrico sérico
  • exames de imagem abdominal
  • exame imagiológico vascular
Full details

Treatment algorithm

ACUTE

adultos não gestantes: baixo risco de trombose sem trombocitose acentuada (contagem plaquetária <1000 × 10⁹/L [<1000 × 10³/microlitro])

adultos não gestantes: alto risco de trombose sem trombocitose acentuada (contagem plaquetária <1000 × 10⁹/L [<1000 × 10³/microlitro])

adultos não gestantes: com trombocitose acentuada (contagem plaquetária ≥1000 × 10⁹/L [≥1000 × 10³/microlitro])

gestante

crianças

ONGOING

adultos não gestantes: alto risco de trombose e intolerantes ou resistentes à terapia citorredutora de primeira e segunda linha

Contributors

Authors

Prithviraj Bose, MD

Professor

Leukemia Department

Division of Cancer Medicine

The University of Texas MD Anderson Cancer Center

Houston

TX

Disclosures

PB has received research support from Incyte, BMS, CTI BioPharma, Cogent BioSciences, Blueprint Medicines, Ionis, Disc Medicine, Morphosys, Kartos, Telios, Sumitomo Pharma, Promedior and NS Pharma, and consulting fees/honoraria from Incyte, BMS, CTI BioPharma, GSK, Abbvie, Morphosys, Cogent BioSciences, Blueprint Medicines, Novartis, Pharma Essentia, and Karyopharm Therapeutics.

Pankit Vachhani, MD

Associate Professor

Division of Hematology/Oncology

Department of Medicine

University of Alabama at Birmingham

Birmingham

AL

Disclosures

PV has received consulting fees/honoraria from Abbvie, Amgen, Blueprint Medicines, Cogent Biosciences, Incyte, CTI BioPharma Corp (now Sobi), GlaxoSmith Kline, Karyopharm, Merck, Novartis, Pfizer, Genentech, Stemline, and MorphoSys. He has received speaking engagement fees from Incyte and Blueprint Medicines.

Acknowledgements

The authors would like to gratefully acknowledge Dr Srdan Verstovsek, Dr Constantine Tam, Dr Scott J. Samuelson, and Dr Josef T. Prchal, previous contributors to this topic.

Disclosures

SV, CT, SJS, and JTP declare that they have no competing interests.

Peer reviewers

Christopher Pechlaner, MD

Associate Professor of Medicine

Innsbruck Medical University

Innsbruck

Austria

Disclosures

CP declares that he has no competing interests.

Ross Levine, MD

Assistant Member

Human Oncology and Pathogenesis Program Leukemia Service

Department of Medicine

Memorial Sloan Kettering Cancer Center

New York

NY

Disclosures

None declared.

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

McMullin MF, Harrison CN, Ali S, et al. A guideline for the diagnosis and management of polycythaemia vera: a British Society for Haematology guideline. Br J Haematol. 2019 Jan;184(2):176-91. Abstract

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

Tefferi A, Barbui T. Polycythemia vera: 2024 update on diagnosis, risk-stratification, and management. Am J Hematol. 2023 Sep;98(9):1465-87.Full text  Abstract

Barbui T, Tefferi A, Vannucchi AM, et al. Philadelphia chromosome-negative classical myeloproliferative neoplasms: revised management recommendations from European LeukemiaNet. Leukemia. 2018 May;32(5):1057-69. Abstract

Vannucchi AM, Barbui T, Cervantes F, et al; ESMO Guidelines Committee. 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

Reference articles

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

  • Differentials

    • Policitemia secundária devido à hipóxia
    • Trombocitemia essencial (TE)
    • Mielofibrose primária
    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
  • padlock-lockedLog in or subscribe to access all of BMJ Best Practice

Use of this content is subject to our disclaimer