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Polycythaemia vera

  • 概述
  • 理论
  • 诊断
  • 治疗
  • 随访
  • 资源
最后审阅： 18 Aug 2025
最后更新： 03 Dec 2024

小结

定义

病史和体格检查

关键诊断因素

  • presence of risk factors
  • features of thrombosis
完整详情

其他诊断因素

  • features of haemorrhage
  • headache
  • generalised weakness/fatigue
  • pruritus
  • night sweats and bone pain
  • erythromelalgia
  • splenomegaly
  • plethora/ruddy cyanosis
  • tinnitus
  • blurry vision
  • arthralgia
  • abdominal discomfort
  • hyperhidrosis (excessive sweating)
完整详情

危险因素

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

诊断性检查

首要检查

  • haemoglobin
  • haematocrit
  • 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
完整详情

需考虑的检查

  • 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
完整详情

治疗流程

急症处理

non-pregnant adults: low risk for thrombosis without marked thrombocytosis (platelet count <1000 × 10⁹/L [<1000 × 10³/microlitre])

non-pregnant adults: high risk for thrombosis without marked thrombocytosis (platelet count <1000 × 10⁹/L [<1000 × 10³/microlitre])

non-pregnant adults: with marked thrombocytosis (platelet count ≥1000 × 10⁹/L [≥1000 × 10³/microlitre])

pregnant

children

持续性治疗

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

撰稿人

作者

Prithviraj Bose, MD

Professor

Leukemia Department

Division of Cancer Medicine

The University of Texas MD Anderson Cancer Center

Houston

TX

利益声明

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

利益声明

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.

鸣谢

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.

利益声明

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

同行评议者

Christopher Pechlaner, MD

Associate Professor of Medicine

Innsbruck Medical University

Innsbruck

Austria

利益声明

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

利益声明

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.

参考文献

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.

关键文献

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. 摘要

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

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

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. 摘要

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.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.

  • 鉴别诊断

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  • 指南

    • 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
    更多 指南
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