What changed: Polycythaemia vera (PV)

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Erythrocytosis in adults with polycythaemia vera

FDA approval for phlebotomy-dependent polycythaemia vera.

Response (no phlebotomy eligibility) weeks 20 to 32: 77% vs 33%.

Rusfertide added to standard care made most patients phlebotomy-free over weeks 20 to 32.

Complete response within 1 year: 43% vs 26%; complete response associated with better event-free survival.

Ruxolitinib beat best available therapy after hydroxyurea failure, and complete responders had better event-free survival.

Polycythaemia vera

Haematocrit at or below 45% without progression at 12 months: 84% vs 60%.

Ropeginterferon plus phlebotomy kept more low-risk patients at target than phlebotomy alone.

By 36 months, complete haematological response with improved disease burden and molecular response were both more frequent with ropeginterferon than hydroxyurea.

Polycythaemia vera without symptomatic splenomegaly

The first interferon licensed for PV, on PROUD-PV and CONTINUATION-PV; FDA approval followed in November 2021.

Haematocrit control at week 28: 62% vs 19%.

Composite response at week 32: 21% vs 1%; haematocrit control 60% vs 20%.

Polycythaemia vera after hydroxyurea

FDA approval in December 2014 for hydroxyurea-resistant or intolerant PV on the RESPONSE trial; EU approval followed in 2015.

Cardiovascular death or major thrombosis 2.

Keeping the haematocrit under 45 percent gives fewer cardiovascular deaths and major clots than a 45 to 50 percent target.

Four groups report the mutation in almost all PV; the first molecular marker for BCR-ABL1-negative MPN and the target for the JAK inhibitors that followed.

Louis Wasserman's cooperative group runs the first randomised PV trials, comparing phlebotomy, radiophosphorus and chlorambucil, and shows the leukaemia risk of alkylating agents.

PV, essential thrombocythaemia, myelofibrosis and CML proposed as related diseases of the marrow.

William Osler collects cases of chronic cyanosis with polycythaemia and splenomegaly.

Louis Henri Vaquez reports a patient with persistent excess red cells and an enlarged spleen.