Polycythemia Vera (PV) is a chronic, BCR::ABL1-negative, myeloproliferative neoplasm characterized by increased hematocrit and platelet/leukocyte counts, as well as by a high risk of thrombosis and progression to myelofibrosis and acute leukemia. The initial discovery of the JAK2V617F driver mutation, followed by a more in-depth knowledge of the mechanisms that regulate inflammation and iron homeostasis, significantly advanced our understanding of PV pathophysiology, paving the way for a shift from symptom-focused vs. biology-focused treatments. PV management is complex and should be individualized, considering age, comorbidities, clinical presentation, disease course, and patient preferences. As such, optimal care requires a dynamic approach involving coordinated collaboration among healthcare professionals, in order to reduce complications, and enhance patients’ quality of life and outcomes. This narrative review explores principles and benefits of integrated care for PV patients, illustrated through two representative clinical cases. We propose that a transdisciplinary approach be adopted throughout the diagnostic and therapeutic continuum for PV to optimize patient outcomes. The review also proposes a novel cytoreductive treatment algorithm in PV going beyond the conventional risk stratification based on patient’s age and history of thrombosis but rather focusing on patient’s characteristics and treatment needs. Finally, we examine ongoing clinical studies and the latest results and insights on PV therapies (including the JAK inhibitor ruxolitinib, the next-generation mono-pegylated type I interferon ropeginterferon alfa-2b, the iron metabolism modifiers and other experimental approaches) that could reshape the standard of care in PV.
Palandri et al. (Sat,) studied this question.