PV is part of the myeloproliferative neoplasms (MPNs) —blood cancers in which a clonal bone marrow stem/progenitor cell gains a growth advantage and drives sustained overproduction of blood cells. The high prevalence of JAK2 mutations in PV supports its clonal myeloid neoplasm biology. [1][2]

Key reasons PV is classified as cancer:

• Clonal stem-cell origin (one “starter” cell in the marrow): PV arises from an altered hematopoietic stem/progenitor cell that expands over time. [2]
• Sustained overproduction: Red blood cells increase, and white blood cells and platelets may also be elevated (panmyelosis). [1][2]
• Risk of evolution: Over years, a minority of patients develop post-PV myelofibrosis or acute myeloid leukemia (AML). Risk is influenced by disease duration, patient factors, and therapy exposure.[5]

These features mirror cancer biology, though PV behaves differently from fast blood cancers or solid tumors.

Despite its classification, many patients—and sometimes clinicians in casual conversation— describe PV as “not really cancer”. This typically reflects the clinical course and lived experience of PV rather than its underlying biology. [4][5]

• Often indolent: PV is generally a chronic, relatively indolent myeloid neoplasm in many patients, with long-term management focused on structured monitoring and complication prevention; however, its course can be interrupted by thrombotic or transformational events [6][7].
• Routine management: For some, periodic phlebotomy and low-dose aspirin can help control hematocrit and reduce complications for long periods. [3][5]
• Survival may be long: In contemporary series, selected patients—especially those diagnosed younger and with good risk-factor control—can have survival approaching population expectations, although this is not universal. [4][5]
• Different treatment goal: Unlike many cancers where cure is the primary aim, PV management typically prioritizes preventing clots, controlling symptoms, and reducing the risk of evolution. [3][5]

In this sense, PV behaves more like a chronic condition than a traditional cancer.

While survival may be reassuring, PV is still symptomatically burdensome. Common features include:

• Fatigue that can feel disproportionate to lab values.
• Aquagenic pruritus (itching after warm showers).
• Microvascular symptoms such as headaches, dizziness, or erythromelalgia.
• Abdominal fullness related to splenomegaly.
• Thrombosis or bleeding events.

Even when survival is long, symptoms and complications can impact quality of life and reinforce the need for individualized, long-term monitoring. [1][2][3]

Large cohort studies and contemporary reviews generally suggest:

• Median survival varies by age and risk profile. Contemporary cohorts suggest substantial heterogeneity, with risk-stratified median survivals spanning roughly the ~11 to ~28-year range; survival can be considerably longer in younger patients. [7][8]
• In selected patients with effective risk management, survival may approach that of the general population. [4]
• Disease evolution can occur: a minority of patients progress to post-PV myelofibrosis or AML over long follow-up. [5]
• Secondary cancer vigilance: higher rates of non-melanoma skin cancer have been reported in PV/ET populations, and some studies note higher risk among patients exposed to hydroxyurea [9]

Overall, PV can be compatible with long survival, but it remains a lifelong neoplasm with ongoing risks that warrant follow-up. [4][5]

1. Is polycythemia vera considered a cancer?

Yes, PV is classified as a chronic blood cancer (myeloproliferative neoplasm). It arises from a clonal stem/progenitor cell and carries long-term risks, including thrombosis and disease evolution, even though it often progresses slowly. [1][2][5]

2. Why do some doctors say PV is “not really cancer”?

Doctors may use that phrase to communicate PV’s typically chronic pace and long-term management approach. PV care often emphasizes clot prevention and symptom control rather than cure-intent therapy. [3][4][5]

3. What is the life expectancy with PV?

It varies by age, cardiovascular risk, and disease features. In selected patients—particularly those diagnosed younger and well controlled—survival may approach that of the general population, but this is not guaranteed. [4][5]

4. Can PV turn into a more aggressive cancer?

Yes, though the risk is relatively low. Over long follow-up, a minority of patients progress to post-PV myelofibrosis or AML; risk is influenced by disease duration, patient factors, and therapy exposure. [5]

5. What is the primary goal of treatment in PV?

A central goal is preventing blood clots, along with controlling hematocrit, relieving symptoms, and monitoring for progression. [3][5]

PV — Polycythemia vera

MPN — Myeloproliferative neoplasm

MPNs — Myeloproliferative neoplasms

JAK2 — Janus kinase 2

AML — Acute myeloid leukemia

PV/ET — Polycythemia vera / Essential thrombocythemia

ET — Essential thrombocythemia

FAQs — Frequently asked questions

1. Spivak, J. L. (2018). Polycythemia vera. Current Treatment Options in Oncology, 19(2), 12.
2. Spivak, J. L. (2002). Polycythemia vera: myths, mechanisms, and management. Blood, 100(13), 4272–4290.
3. Tefferi, A., Vannucchi, A. M., & Barbui, T. (2018). Polycythemia vera treatment algorithm 2018. Blood Cancer Journal, 8(1), 3.
4. Tefferi, A., Vannucchi, A. M., & Barbui, T. (2021). Polycythemia vera: historical oversights, diagnostic details, and therapeutic views. Leukemia, 35(12), 3339–3351. https://doi.org/10.1038/s41375-021-01401-3
5. Tefferi, A., & Barbui, T. (2020). Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk stratification and management. Am J Hematol, 95(12), 1599–1613.
6. Tefferi, A., Vannucchi, A. M., & Barbui, T. (2021). Polycythemia vera: historical oversights, diagnostic details, and therapeutic views. Leukemia, 35(12), 3339–3351. https://doi.org/10.1038/s41375-021-01401-3
7. Tefferi, A., & Barbui, T. (2023). Polycythemia vera: 2024 update on diagnosis, risk-stratification, and management. American journal of hematology, 98(9), 1465–1487. https://doi.org/10.1002/ajh.27002
8. Tefferi, A., Rumi, E., Finazzi, G., Gisslinger, H., Vannucchi, A. M., Rodeghiero, F., Randi, M. L., Vaidya, R., Cazzola, M., Rambaldi, A., Gisslinger, B., Pieri, L., Ruggeri, M., Bertozzi, I., Sulai, N. H., Casetti, I., Carobbio, A., Jeryczynski, G., Larson, D. R., Müllauer, L., … Barbui, T. (2013). Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study. Leukemia, 27(9), 1874–1881. https://doi.org/10.1038/leu.2013.163
9. Tefferi, A., Vannucchi, A. M., & Barbui, T. (2018). Polycythemia vera treatment algorithm 2018. Blood Cancer Journal, 8(1), 3.