Resumen
Definición
Anamnesis y examen
Principales factores de diagnóstico
- features of thrombosis
Otros factores de diagnóstico
- 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)
Factores de riesgo
- age >60 years
- history of Budd-Chiari syndrome (BCS)
- affected family member
- Janus kinase 2 (JAK2) mutations (JAK2 V617F and JAK2 exon 12)
Pruebas diagnósticas
Primeras pruebas diagnósticas para solicitar
- 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
Pruebas diagnósticas que deben considerarse
- oxygen saturation
- red blood cell (RBC) mass
- further mutational testing
- cytogenetic and molecular testing: BCR::ABL1
- serum uric acid
- serum LDH
Algoritmo de tratamiento
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
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
Divulgaciones
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
Divulgaciones
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.
Agradecimientos
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.
Divulgaciones
SV, CT, SJS, and JTP declare that they have no competing interests.
Revisores por pares
Christopher Pechlaner, MD
Associate Professor of Medicine
Innsbruck Medical University
Innsbruck
Austria
Divulgaciones
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
Divulgaciones
None declared.
Agradecimiento de los revisores por pares
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Divulgaciones
Las afiliaciones y divulgaciones de los revisores por pares se refieren al momento de la revisión.
Referencias
Artículos principales
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. Resumen
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 Resumen
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. Resumen
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 Resumen
Artículos de referencia
Una lista completa de las fuentes a las que se hace referencia en este tema está disponible para los usuarios con acceso a todo BMJ Best Practice.
Diferenciales
- Secondary polycythemia owing to hypoxia
- Essential thrombocythemia (ET)
- Primary myelofibrosis
Más DiferencialesGuías de práctica clínica
- 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
Más Guías de práctica clínica
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