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 transformation to myelofibrosis or secondary acute myeloid leukemia include:
- Older age
- Extended disease duration
- Leukocytosis
- Exposure to phosphorus-32, pipobroman, or chlorambucil
The risk factors for the development of myelofibrosis from polycythemia vera include[2]:
- Increased age
- Leukocytosis
- High variant allele frequency of the JAK2 mutation (high allele burden)
- Thrombocytosis
- Splenomegaly
The risk factors for the development of acute myeloid leukemia from polycythemia vera include[2]:
- Increased age
- Leukocytosis
- Splenomegaly
- 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.
- ↑ 2.0 2.1 2.2 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.