In patient-facing terms, “advanced/progressed PV” is commonly used to describe one or more of the following:

• Evolution to post-PV myelofibrosis, based on consensus diagnostic criteria. [3,5]
• Leukemic transformation (blast phase/AML), which is uncommon but serious. [3]

Increasing symptom burden or complications despite treatment, prompting reassessment of disease status and management. [1]

Symptoms vary by person and by whether PV has evolved to myelofibrosis or blast phase. Commonly reported issues include:

1) Severe fatigue and constitutional symptoms

Fatigue is common in PV and may become more pronounced with disease evolution. [6,7] Some people also report night sweats, unintentional weight loss, or fevers (constitutional symptoms). [4]

2) Enlarging spleen (splenomegaly) and early satiety

Splenomegaly can cause left-upper abdominal discomfort and early satiety and is a key clinical feature targeted in myelofibrosis-phase management. [4]

3) Low blood counts (more consistent with myelofibrosis features)

With evolution to post-PV myelofibrosis, anemia and thrombocytopenia may occur and should be evaluated by the treating hematology team. [4]

4) If transformation to blast phase/AML occurs

Blast phase/AML typically involves rapidly worsening marrow failure manifestations (fatigue/anemia, infections, bruising/bleeding) and requires urgent specialist care. [3]

Management is individualized and may combine disease-directed therapy and supportive care. Supportive approaches may include:

• Treating anemia-related symptoms. [4]
• Addressing splenic symptoms and overall symptom burden with clinician-directed therapies.

Palliative care note: Palliative care focuses on symptom relief, coping, and quality of life and can be used alongside active treatment; oncology guidance supports early integration when symptom burden or quality-of-life needs are significant. [8]

1. How does advanced/progressed PV differ from earlier disease?

Earlier PV is often characterized by elevated blood counts and management focused on reducing thrombotic risk and controlling counts/symptoms. If PV evolves to post-PV myelofibrosis, features can shift toward marrow fibrosis with enlarging spleen, constitutional symptoms, and falling blood counts (e.g., anemia and/or thrombocytopenia). [1,2,4]

2. Does everyone with PV develop advanced/progressed disease?

No. PV is often managed long-term with ongoing monitoring. Some patients progress to post-PV myelofibrosis, and a smaller subset transform to blast phase/AML. [2,3]

3. How important is palliative care in advanced/progressed PV?

Palliative care is supportive care focused on symptom relief, coping, and quality of life, and it can be delivered alongside disease-directed treatment. ASCO’s guideline update recommends palliative care integration for patients with significant symptom burden and quality-of-life needs. [9]

PV — Polycythemia vera

JAK2 — Janus kinase 2

AML — Acute myeloid leukemia

ASCO — American Society of Clinical Oncology

FAQs — Frequently asked questions

1. Marchetti, M., Vannucchi, A. M., Griesshammer, M., Harrison, C., Koschmieder, S., Gisslinger, H., Álvarez-Larrán, A., De Stefano, V., Guglielmelli, P., Palandri, F., Passamonti, F., Barosi, G., Silver, R. T., Hehlmann, R., Kiladjian, J.-J., & Barbui, T. (2022). Appropriate management of polycythaemia vera with cytoreductive drug therapy: European LeukemiaNet 2021 recommendations. The Lancet Haematology, 9(4), e301–e311. https://doi.org/10.1016/S2352-3026(22)00046-1
2. 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
3. Tefferi, A., Alkhateeb, H., & Gangat, N. (2023). Blast phase myeloproliferative neoplasm: Contemporary review and 2024 treatment algorithm. Blood Cancer Journal, 13(1), 108. https://doi.org/10.1038/s41408-023-00878-8
4. Tefferi, A. (2023). Primary myelofibrosis: 2023 update on diagnosis, risk-stratification, and management. American Journal of Hematology, 98(5), 801–821. https://doi.org/10.1002/ajh.26857
5. Barosi, G., Mesa, R. A., Thiele, J., Cervantes, F., Campbell, P. J., Verstovsek, S., Dupriez, B., Levine, R. L., Passamonti, F., Gotlib, J., Reilly, J. T., Vannucchi, A. M., Hanson, C. A., Solberg, L. A., Orazi, A., & Tefferi, A. (2008). Proposed criteria for the diagnosis of post-polycythemia vera and post-essential thrombocythemia myelofibrosis: A consensus statement from the International Working Group for Myelofibrosis Research and Treatment. Leukemia, 22(2), 437–438. https://doi.org/10.1038/sj.leu.2404914
6. Cuthbert, D., & Stein, B. L. (2019). Polycythemia vera-associated complications: Pathogenesis, clinical manifestations, and effects on outcomes. Journal of Blood Medicine, 10, 359–371. https://doi.org/10.2147/JBM.S189922
7. Edahiro, Y., Kirito, K., Gotoh, A., Takenaka, K., et al. (2023). P1060: The impact of polycythemia vera on daily living, and symptom and treatment perception gaps between patients and physicians: Results of a cross-sectional survey in Japan. HemaSphere, 7(Suppl), e5917801. https://doi.org/10.1097/01.HS9.0000971136.59178.01
8. Sanders, J. J., Temin, S., Ghoshal, A., Alesi, E. R., Ali, Z. V., Chauhan, C., Cleary, J. F., Epstein, A. S., Firn, J. I., Jones, J. A., Litzow, M. R., Lundquist, D., Mardones, M. A. M., Nipp, R. D., Rabow, M. W., Rosa, W. E., Zimmermann, C., & Ferrell, B. R. (2024). Palliative care for patients with cancer: ASCO guideline update. Journal of Clinical Oncology, 42(19), 2336–2357. https://doi.org/10.1200/JCO.24.00542