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9 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Polycythaemia vera is a slow blood cancer in which a single faulty gene, JAK2, makes the bone marrow produce too many red cells. Thick blood causes clots, so treatment thins it (blood removal, aspirin) and, for higher-risk patients, calms the marrow with hydroxyurea, interferon or ruxolitinib; a hepcidin mimic, rusfertide, now controls red cell counts without regular blood removal.
Polycythaemia vera is one of the classical myeloproliferative neoplasms. Almost every case carries a mutation in JAK2 (V617F in about 95 percent, exon 12 in most of the rest) that keeps the red-cell growth signal switched on without erythropoietin. The result is a high haematocrit, often with raised platelets and white cells, an enlarged spleen, itching after warm water, burning red hands and feet, and above all a raised risk of arterial and venous thrombosis, including clots in unusual places such as the hepatic veins. Diagnosis rests on the blood count, the JAK2 mutation, a hypercellular marrow and a low erythropoietin level. Treatment is risk-adapted: everyone has low-dose aspirin and phlebotomy to keep the haematocrit under 45 percent, a target proven by the CYTO-PV trial; people over 60 or with a prior clot add a cytoreductive drug, hydroxyurea or ropeginterferon alfa-2b, with ruxolitinib for those hydroxyurea fails. Rusfertide, a hepcidin mimetic that starves red-cell production of iron, was approved in 2026 for phlebotomy-dependent disease. Over decades a minority progress to post-PV myelofibrosis and a few percent to acute leukaemia, which is why the field is now chasing molecular remission with interferon and JAK2 V617F-selective inhibitors.
| Setting | Approach | Guideline |
|---|---|---|
| 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. | not mapped |
| All patients | Low-dose aspirin unless contraindicated, phlebotomy to a haematocrit under 45 percent (CYTO-PV), and control of cardiovascular risk factors. | not mapped |
| 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). | not mapped |
| 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. | not mapped |
| Hydroxyurea resistance or intolerance | Ruxolitinib (RESPONSE, RESPONSE-2, MAJIC-PV) for haematocrit control, spleen shrinkage and symptom relief; interferon if not already tried. | not mapped |
| 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). | not mapped |
| Itching and burning extremities | Antihistamines, aspirin for erythromelalgia, interferon or ruxolitinib for severe aquagenic pruritus. | not mapped |
| Post-PV myelofibrosis | Managed as myelofibrosis: JAK inhibitors for spleen and symptoms, momelotinib for anaemia, allogeneic stem cell transplant for fit higher-risk patients. | not mapped |