Appointment sheet
Polycythaemia vera (PV)
Prepared with OnCo (onco.cc/prep/polycythaemia-vera/). Orientation, not medical advice; your team knows your case.
My details
Name
Date of appointment
Hospital and clinician
Who is coming with me
What I know, what is unclear, changes to discuss
Saved in this browserWhat I know so far
What is unclear to me
Changes since last time
My questions
28 on the sheetNewly diagnosed
- 1.What is my exact diagnosis, stage, and grade, and which tests established them?
- 2.Which biomarkers have been tested on my tumour (for example JAK2 V617F allele burden, JAK2 exon 12 mutations, Haematocrit, with treatment targeting below 45 percent, Serum erythropoietin, Leukocyte count above 11 x 10^9/L), and what were the results?
- 3.Which subtype is my cancer, and does that change the recommended treatment?
- 4.Is germline (inherited) genetic testing recommended for me or my family?
Diagnosis
- 5.For my situation (diagnosis), which of the standard options do you recommend and why?
All patients
- 6.For my situation (all patients), which of the standard options do you recommend and why?
- 7.Am I a candidate for Aspirin, and what side effects should I expect?
- 8.How do the results of CYTO-PV apply to someone like me?
Low risk (under 60, no prior clot)
- 9.For my situation (low risk (under 60, no prior clot)), which of the standard options do you recommend and why?
- 10.Am I a candidate for Ropeginterferon alfa-2b, and what side effects should I expect?
- 11.How do the results of Low-PV apply to someone like me?
High risk (over 60 or prior clot)
- 12.For my situation (high risk (over 60 or prior clot)), which of the standard options do you recommend and why?
- 13.Am I a candidate for Hydroxyurea (hydroxycarbamide), Ropeginterferon alfa-2b, and what side effects should I expect?
- 14.How do the results of PROUD-PV and CONTINUATION-PV apply to someone like me?
Hydroxyurea resistance or intolerance
- 15.For my situation (hydroxyurea resistance or intolerance), which of the standard options do you recommend and why?
- 16.Am I a candidate for Ruxolitinib, and what side effects should I expect?
- 17.How do the results of RESPONSE and RESPONSE-2 apply to someone like me?
Phlebotomy-dependent disease
- 18.For my situation (phlebotomy-dependent disease), which of the standard options do you recommend and why?
- 19.Am I a candidate for Rusfertide, and what side effects should I expect?
- 20.How do the results of VERIFY apply to someone like me?
Itching and burning extremities
- 21.For my situation (itching and burning extremities), which of the standard options do you recommend and why?
Post-PV myelofibrosis
- 22.For my situation (post-pv myelofibrosis), which of the standard options do you recommend and why?
- 23.Am I a candidate for Ruxolitinib, Momelotinib, and what side effects should I expect?
Any stage
- 24.Are there clinical trials I could join, for example of Givinostat, Bomedemstat, Study on Efficacy and Safety of Givinostat Versus Hydroxyurea in Patients With Polycythemia Vera, Clearing the JAK2 clone in polycythaemia vera: interferon plus mutant-selective inhibitors as a route to treatment-free remission?
- 25.Would a second opinion at a high-volume centre change anything, and can you help arrange it?
- 26.What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
- 27.I read that “No treatment has yet been shown to prevent progression to myelofibrosis or leukaemia; interferon's molecular responses are the strongest hint”. How does that affect my plan?
- 28.I read that “Risk stratification still rests on age and clot history; leukocyte count, allele burden and additional mutations are not yet built into treatment decisions”. How does that affect my plan?
The words I may hear
- Phlebotomy (venesection): Removing about a pint of blood through a vein, as in a blood donation, to bring the red cell count down.
- Haematocrit: The share of blood volume made up of red cells.
- Hepcidin: The liver hormone that controls how much iron the body absorbs and releases.
- Erythrocytosis (primary vs secondary): Too many red cells.
- Aquagenic pruritus: Intense itching, prickling or burning of the skin within minutes of contact with water, typically after a shower.
- 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.
Tests and results to bring
Diagnosis: Full blood count, JAK2 V617F and exon 12 testing, serum erythropoietin, and bone marrow biopsy showing trilineage growth; WHO criteria (haemoglobin above 16.5 g/dL in men or 16.0 in women, or haematocrit above 49 or 48 percent). Secondary causes of a high red count (smoking, sleep apnoea, kidney or liver tumours, testosterone) are excluded first.
Biomarker results to ask for: JAK2 V617F allele burden (falls with interferon; a marker of molecular response), JAK2 exon 12 mutations, Haematocrit, with treatment targeting below 45 percent, Serum erythropoietin (low in PV, high in secondary erythrocytosis), Leukocyte count above 11 x 10^9/L (thrombosis risk), Additional mutations in TET2, ASXL1, SRSF2, IDH1/2 (progression risk), Age over 60 and prior thrombosis (the two risk factors that define high-risk disease).
Bring copies of scan reports, pathology and blood results, and a list of every medicine and supplement.
The treatments I may be offered
- Low risk (under 60, no prior clot): Aspirin and phlebotomy alone; ropeginterferon alfa-2b is an option when phlebotomy is poorly tolerated or symptoms persist (Low-PV). (Ropeginterferon alfa-2b, Low-PV)
- High risk (over 60 or prior clot): Add cytoreduction: hydroxyurea, or ropeginterferon alfa-2b (PROUD-PV and CONTINUATION-PV), the latter preferred in younger patients and in pregnancy. (Hydroxyurea (hydroxycarbamide), Ropeginterferon alfa-2b, PROUD-PV and CONTINUATION-PV)
- All patients: Low-dose aspirin unless contraindicated, phlebotomy to a haematocrit under 45 percent (CYTO-PV), and control of cardiovascular risk factors. (Aspirin, CYTO-PV, Phlebotomy (venesection), Haematocrit)
- Phlebotomy-dependent disease: Rusfertide, a hepcidin mimetic given by weekly injection, keeps the haematocrit under 45 percent and removes the need for phlebotomy in most patients (VERIFY). (Rusfertide, VERIFY, Hepcidin)
- Itching and burning extremities: Antihistamines, aspirin for erythromelalgia, interferon or ruxolitinib for severe aquagenic pruritus. (Aquagenic pruritus, Erythromelalgia)
- Post-PV myelofibrosis: Managed as myelofibrosis: JAK inhibitors for spleen and symptoms, momelotinib for anaemia, allogeneic stem cell transplant for fit higher-risk patients. (Myeloproliferative neoplasms (PV, ET, myelofibrosis), Ruxolitinib, Momelotinib, Post-PV myelofibrosis (spent phase))
- Hydroxyurea resistance or intolerance: Ruxolitinib (RESPONSE, RESPONSE-2, MAJIC-PV) for haematocrit control, spleen shrinkage and symptom relief; interferon if not already tried. (Ruxolitinib, RESPONSE, RESPONSE-2, MAJIC-PV)
From the standard of care recorded for this cancer; which apply depends on your stage and biomarkers. Ask which the team recommends and why.
Answers and next steps
Saved in this browserWhat I was told
Agreed next steps, dates and who to call