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Polycythemia vera

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Последний просмотренный: 9 Dec 2025
Last updated: 02 Jan 2026

Резюме

Определение

História e exame físico

Principais fatores diagnósticos

  • features of thrombosis
Detalhes completos

Outros fatores diagnósticos

  • 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)
Detalhes completos

Fatores de risco

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

Investigações diagnósticas

Primeiras investigações a serem solicitadas

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

Investigações a serem consideradas

  • oxygen saturation
  • red blood cell (RBC) mass
  • further mutational testing
  • cytogenetic and molecular testing: BCR::ABL1
  • serum uric acid
  • serum LDH
Detalhes completos

Algoritmo de tratamento

AGUDA

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

CONTÍNUA

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

Colaboradores

Autores

Prithviraj Bose, MD

Professor

Leukemia Department

Division of Cancer Medicine

The University of Texas MD Anderson Cancer Center

Houston

TX

Declarações

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

Declarações

PV has received consulting fees/honoraria for advisory roles from Abbvie, Amgen, Blueprint Medicines, Cogent Biosciences, DISC medicine, Incyte, Sobi, Genentech, Geron, GlaxoSmith Kline, Karyopharm, Merck, MorphoSys, Novartis, Pfizer, Stemline, Servier, Takeda.

Agradecimentos

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.

Declarações

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

Revisores

Christopher Pechlaner, MD

Associate Professor of Medicine

Innsbruck Medical University

Innsbruck

Austria

Declarações

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

Declarações

None declared.

Créditos aos pareceristas

Os tópicos do BMJ Best Practice são constantemente atualizados, seguindo os desenvolvimentos das evidências e das diretrizes. Os pareceristas aqui listados revisaram o conteúdo pelo menos uma vez durante a história do tópico.

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Referências

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Principais artigos

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. Resumo

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

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

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. Resumo

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.Texto completo  Resumo

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

  • Diagnósticos diferenciais

    • Secondary polycythemia owing to hypoxia
    • Essential thrombocythemia (ET)
    • Primary myelofibrosis
    Mais Diagnósticos diferenciais

  • Diretrizes

    • 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
    Mais Diretrizes
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