BSH 2024 | The changing approaches to polycythemia vera: a clinical perspective

So, from a clinical perspective, the diagnosis of polycythemia vera has become much easier with the description, more than a decade ago, of JAK2 mutations. That means we’re picking up patients with unusual presentations, now increasingly, perhaps, picking up patients with a very low level of JAK2. But the approach to management is really largely: assess the risk; that includes vascular risk (We’re a bit bad at doing that in our routine practice, so call out to everyone to think about that.); use aspirin; phlebotomy, or venesection to target the hematocrit generally to 0.45 (very important); and then for higher-risk patients – that would be age over 60 to 65, prior thrombosis – use a drug. And increasingly that higher-risk spectrum is being broadened out to white count more than 11, vascular risk factors, big spleen, unresponsive symptoms. So we’re broadening a bit our high-risk group, and that’s where we’re testing now ruxolitinib in the frontline setting. But I think increasingly, our approach to PV is being a little bit turned on its head to think we should be going earlier in the treatment paradigm because we now have data that reducing the amount of JAK2 or the JAK2 VAF leads to survival advantage for our patients. That came from the MAJIC study and other studies. Now we’re thinking should we be treating patients earlier? And that’s coupled with some big cohorts, including some from ourselves in the European Strategic Working Group, showing that younger patients treated early with interferon have much better myelofibrosis-free survival. So, changes in the field, integration of molecular response, and earlier treatment as well as trials. So really busy in PV.