Polycythemia vera risk factors
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamad Alkateb, MBBCh [2]; Shyam Patel [3]
Overview
Common risk factors in the development of polycythemia vera are old age (65 year old and older), family history, and Ashkenazi Jewish descent. After a patient is diagnosed with polycythemia vera, risk stratification involves assessment of age and thrombotic history. The risk assessment for development of post-PV myelofibrosis or post-PV acute myeloid leukemia include a variety of factors.
Risk Factors
Risk factors in the development of polycythemia vera are:[1]
- Old age (65 years older): The reason for age being a risk factor is that mutations can accumulate in hematopoietic stem cells over time, resulting in a higher probability of a JAK2 mutation.
- Family history of polycythemia vera: First-degree relatives of patients with polycythemia vera have a 5 to 7-fold higher risk for development of a myeloproliferative neoplasm. The cumulative risk of developing myelofibrosis is 6% at 10 years, 14% at 15 years, and 26% at 20 years from the initial diagnosis of polycythemia vera.[2]
- Ashkenazi Jewish descent: This population has a slightly higher risk for developing polycythemia vera
Risk Stratification
High risk features of polycythemia vera include:
- Old age
- History of thrombotic event
- Presence of JAK2 mutation
The risk factors for the development of thrombosis include:[3]
- Age >60 years
- History of prior thrombosis
- Leukocytosis
- Increased JAK2 V617F allele burden
- High risk gene expression profile
The risk factors for the transformation to myelofibrosis or secondary acute myeloid leukemia include:[3]
- Older age
- Extended disease duration
- Leukocytosis
- Exposure to phosphorus-32, pipobroman, or chlorambucil
The risk factors associated with decreased survival include:[3]
- Older age
- Leukocytosis
- History of venous thrombosis
- Abnormal karyotype
References
- ↑ Barbui T, Carobbio A, Rumi E, Finazzi G, Gisslinger H, Rodeghiero F; et al. (2014). "In contemporary patients with polycythemia vera, rates of thrombosis and risk factors delineate a new clinical epidemiology". Blood. 124 (19): 3021–3. doi:10.1182/blood-2014-07-591610. PMID 25377561.
- ↑ Vannucchi AM (2017). "From leeches to personalized medicine: evolving concepts in the management of polycythemia vera". Haematologica. 102 (1): 18–29. doi:10.3324/haematol.2015.129155. PMC 5210229. PMID 27884974.
- ↑ 3.0 3.1 3.2 Stein BL, Oh ST, Berenzon D, Hobbs GS, Kremyanskaya M, Rampal RK, Abboud CN, Adler K, Heaney ML, Jabbour EJ, Komrokji RS, Moliterno AR, Ritchie EK, Rice L, Mascarenhas J, Hoffman R (November 2015). "Polycythemia Vera: An Appraisal of the Biology and Management 10 Years After the Discovery of JAK2 V617F". J. Clin. Oncol. 33 (33): 3953–60. doi:10.1200/JCO.2015.61.6474. PMC 4979103. PMID 26324368.