Polycythaemia Vera: Management

Key points

• PV: a myeloproliferative disorder — clonal marrow stem-cell proliferation raising red cell mass (with variable neutrophils/platelets); peak in the sixth decade.
• Diagnosis: JAK2 V617F mutation in about 95% (JAK2 exon 12 in most of the rest), raised red cell mass, low serum erythropoietin; features include hyperviscosity, aquagenic pruritus, splenomegaly and erythromelalgia.
• First-line management: low-dose aspirin + venesection (target haematocrit under 0.45).
• Cytoreduction (higher-risk or poorly tolerated venesection): hydroxycarbamide first-line; ruxolitinib (a JAK inhibitor) for hydroxycarbamide-resistant/intolerant disease; interferon-alfa (especially younger patients or pregnancy). Phosphorus-32 is now rarely used.
• Prognosis: thrombosis is the major cause of morbidity/mortality; about 5-15% progress to myelofibrosis and 5-15% to acute leukaemia (risk increased by some cytotoxics).

Decision points in this pathway

• High risk / cytoreduction needed?

The full step-by-step algorithm, including what to do at each branch, opens in the app for approved members.