Polycythemia vera risk factors

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ifeoma Odukwe, M.D. [2] Mohamad Alkateb, MBBCh [3] Shyam Patel [4]

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 in the development of polycythemia vera are:^[1]

• Old age (65 years older)
  • 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

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

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