The first 60 days: Myeloproliferative neoplasms (PV, ET, myelofibrosis)
Myeloproliferative neoplasms are slow-growing blood cancers in which the marrow overproduces red cells (polycythaemia vera), platelets (essential thrombocythaemia) or scar tissue (myelofibrosis). Almost all carry a mutation in JAK2, CALR or MPL; treatment aims to prevent clots and control symptoms, and only transplant cures myelofibrosis. Below, week by week, is what OnCo's record of Myeloproliferative neoplasms (PV, ET, myelofibrosis) says about the first two months: the order is typical, the timing is yours to ask about. Sections appear only where the record has something to say. Orientation, not medical advice.
What happens now
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
- Medical oncologistNamed in the standard of care for: PV, ET, Myelofibrosis, intermediate-2/high risk, Anaemia of myelofibrosis.
- Transplant and cell therapy teamNamed in the standard of care for: PV, Myelofibrosis, intermediate-2/high risk.
- Palliative and supportive care teamNamed in the standard of care for: Anaemia of myelofibrosis.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
- 1.Myelofibrosis, intermediate-2/high riskNCCN category Category 1 (ruxolitinib, fedratinib); 2A (pacritinib, momelotinib), NCCN Guidelines: MPN
JAK inhibitor for spleen and symptoms: ruxolitinib (COMFORT), fedratinib, pacritinib if platelets <50×10⁹/L, momelotinib if anaemic (MOMENTUM); allogeneic HSCT for eligible patients (the only cure).
Low-dose aspirin, phlebotomy to haematocrit <45%; cytoreduction (hydroxyurea or ropeginterferon alfa-2b) for high-risk; ruxolitinib after hydroxyurea failure (RESPONSE); rusfertide to eliminate phlebotomy need (VERIFY, 2026).
Risk-adapted (IPSET-thrombosis): observation or aspirin in low risk; hydroxyurea or interferon in high risk; anagrelide second line.
Momelotinib, luspatercept (INDEPENDENCE), ESA, danazol, transfusion.
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example JAK2 V617F and exon 12, CALR type 1/2, MPL W515, High-molecular-risk mutations, DIPSS-plus / MIPSS70+ v2.0), and what were the results?These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Recognised subtypes for this cancer include Polycythaemia vera, Essential thrombocythaemia, Prefibrotic and overt primary myelofibrosis.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
PV
- For my situation (pv), which of the standard options do you recommend and why?Guideline options include: Low-dose aspirin, phlebotomy to haematocrit <45%; cytoreduction (hydroxyurea or ropeginterferon alfa-2b) for high-risk; ruxolitinib after hydroxyurea failure (RESPONSE); rusfertide to eliminate phlebotomy need (VERIFY, 2026).
- Am I a candidate for Hydroxyurea (hydroxycarbamide), Ropeginterferon alfa-2b, Ruxolitinib or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
ET
- For my situation (et), which of the standard options do you recommend and why?Guideline options include: Risk-adapted (IPSET-thrombosis): observation or aspirin in low risk; hydroxyurea or interferon in high risk; anagrelide second line.
- Am I a candidate for Hydroxyurea (hydroxycarbamide), Ropeginterferon alfa-2b, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Myelofibrosis, intermediate-2/high risk
- For my situation (myelofibrosis, intermediate-2/high risk), which of the standard options do you recommend and why?Guideline options include: JAK inhibitor for spleen and symptoms: ruxolitinib (COMFORT), fedratinib, pacritinib if platelets <50×10⁹/L, momelotinib if anaemic (MOMENTUM); allogeneic HSCT for eligible patients (the only cure).
- Am I a candidate for Ruxolitinib, Fedratinib, Pacritinib or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Anaemia of myelofibrosis
- For my situation (anaemia of myelofibrosis), which of the standard options do you recommend and why?Guideline options include: Momelotinib, luspatercept (INDEPENDENCE), ESA, danazol, transfusion.
- Am I a candidate for Momelotinib, Luspatercept, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Are there clinical trials I could join, for example of Rusfertide, Momelotinib, Luspatercept, Allogeneic stem cell transplantation?Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Supportive care improves quality of life and helps patients complete treatment.
- I read that “No disease-modifying therapy in myelofibrosis short of transplant”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “MPN in blast phase: outcomes as poor as secondary AML”. How does that affect my plan?Open problems are where trials and second opinions matter most.
Trials open now for this cancer in OnCo, largest phase first. Joining a trial is a decision like any other: ask what the comparison arm is, whether a placebo is used, and what happens if you leave. The cancer page searches ClinicalTrials.gov live for more.
- A Phase 3 Study of Rusfertide in Patients With Polycythemia VeraPhase 3 · active · NCT05210790A Phase 3 Study of the Hepcidin Mimetic Rusfertide (PTG-300) in Patients With Polycythemia Vera
- A Study Comparing Imetelstat Versus Best Available Therapy for the Treatment of Intermediate-2 or High-risk Myelofibrosis (MF) Who Have Not Responded to Janus Kinase (JAK)-Inhibitor TreatmentPhase 3 · active · NCT04576156A Randomized Open-Label, Phase 3 Study to Evaluate Imetelstat (GRN163L) Versus Best Available Therapy (BAT) in Patients With Intermediate-2 or High-risk Myelofibrosis (MF) Relapsed / Refractory (R/R) to Janus Kinase (JAK) Inhibitor
- A Study to Assess Efficacy, Safety, and Tolerability of P1101 in Adult Patients With PVPhase 3 · active · NCT05481151A Phase IIIb, Randomized, Open-Label, Parallel Group, Multicenter Study to Assess Efficacy, Safety, and Tolerability of Two Dosing Regimens of Ropeginterferon Alfa-2b-njft (P1101) in Adult Patients With Polycythemia Vera (PV)
- A Study to Evaluate Long-term Safety in Participants Who Have Participated in Other Luspatercept (ACE-536) Clinical TrialsPhase 3 · recruiting · NCT04064060A Phase 3b, Open-label, Single-arm, Rollover Study to Evaluate Long-term Safety in Subjects Who Have Participated in Other Luspatercept (ACE-536) Clinical Trials
- A Study to Evaluate Safety and Efficacy of Bomedemstat (MK-3543-017)Phase 3 · recruiting · NCT06351631A Multicenter, Open-Label, Extension Study Evaluating the Safety and Efficacy of Bomedemstat for the Treatment of Participants Enrolled in a Prior Bomedemstat Clinical Study
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Myeloproliferative neoplasms (PV, ET, myelofibrosis): the full pageMyeloproliferative neoplasms are slow-growing blood cancers in which the marrow overproduces red cells (polycythaemia vera), platelets (essential thrombocythaemia) or scar tissue (myelofibrosis). Almost all carry a mutation in JAK2, CALR or MPL; treatment aims to prevent clots and control symptoms, and only transplant cures myelofibrosis.
- One-page appointment sheetYour questions, the words you may hear, what to bring, and space for the answers. Print it.
- Treatment sequencingWhich treatment tends to follow which, line by line.
- NavigatorStandard of care for your stage, what you have tried, and trials near you.
- Aquagenic pruritus: Intense itching, prickling or burning of the skin within minutes of contact with water, typically after a shower.
- ELN 2022 risk classification: The three-tier system (favourable, intermediate, adverse) that decides how aggressively an adult with AML is treated and whether a transplant is recommended.
- JAK2 V617F: A single letter change in the JAK2 gene that jams the growth signal for blood cells in the on position.
- Post-PV myelofibrosis (spent phase): The late stage some people with polycythaemia vera reach after many years, when the marrow scars over, the red count falls and the spleen swells.
- Erythromelalgia: Burning pain, redness and heat in the hands or feet, brought on by warmth.
Every term links to the glossary.