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Polycythemia vera

  • Overview
  • Theory
  • Diagnosis
  • Management
  • Follow up
  • Resources
Last reviewed: 14 Aug 2025
Last updated: 03 Dec 2024

Summary

Definition

History and exam

Key diagnostic factors

  • features of thrombosis
Full details

Other diagnostic factors

  • features of hemorrhage
  • headache
  • generalized weakness/fatigue
  • pruritus
  • night sweats and bone pain
  • erythromelalgia
  • splenomegaly
  • plethora/ruddy cyanosis
  • tinnitus
  • blurry vision
  • arthralgia
  • abdominal discomfort
  • hyperhidrosis (excessive sweating)
Full details

Risk factors

  • age >60 years
  • history of Budd-Chiari syndrome (BCS)
  • affected family member
  • Janus kinase 2 (JAK2) mutations (JAK2 V617F and JAK2 exon 12)
Full details

Diagnostic tests

1st tests to order

  • hemoglobin
  • hematocrit
  • white blood cell (WBC) count
  • platelet count
  • mean corpuscular volume (MCV)
  • peripheral blood smear
  • liver function tests (LFTs)
  • serum ferritin
  • JAK2 gene mutation testing
  • bone marrow biopsy
Full details

Tests to consider

  • oxygen saturation
  • serum erythropoietin
  • red blood cell (RBC) mass
  • further mutational testing
  • cytogenetic and molecular testing: BCR::ABL1
  • serum uric acid
  • abdominal imaging
  • vascular imaging
Full details

Treatment algorithm

ACUTE

nonpregnant adults: low risk for thrombosis without marked thrombocytosis (platelet count <1000 × 10³/microliter [<1000 × 10⁹/L])

nonpregnant adults: high risk for thrombosis without marked thrombocytosis (platelet count <1000 × 10³/microliter [<1000 × 10⁹/L])

nonpregnant adults: with marked thrombocytosis (platelet count ≥1000 × 10³/microliter [≥1000 × 10⁹/L])

pregnant

children

ONGOING

nonpregnant adults: high risk of thrombosis and intolerant or resistant to first- and second-line cytoreductive therapy

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

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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.

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  • 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
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