Polycythemia Vera in Patients Aged > 80 Years: Real-Life Experience of the NPM Ph Negative Latial Group

Background: Increasing life expectancy has lead to a progressive increment in the proportion of patients (pts) with myeloproliferative neoplasms (MPN) aged ≥ 80 years at diagnosis. However, so far, few studies have evaluated very elderly pts with polycythemia vera (PV) Aim: To describe the clinical features and the course of disease in a real-life cohort of PV pts aged ≥80 years Methods: From 1/2000 to 12/2023, 100 consecutive pts aged ≥ 80 years were diagnosed in 7 hematologic Centres and enrolled in the retrospective and prospective databases of the Latial group for Ph-negative MPN. Diagnosis was revised according to WHO 2022 criteria. Results: Main features at diagnosis were as follows: M/F 42/58, median age 83.1 years [interquartile range (IQR) 81.3 - 85.9], median Hb value 17.5 g/dl (IQR 16.0 - 18.5), median Ht 53.7% (IQR 50.5 - 57.9), median WBC count 10.2 x 10⁹/l (IQR 8.7 - 13.5), median PLT count 520 x 10⁹/l (IQR 370 - 690).Marrow biopsy was performed in 13/89 evaluable cases (14.5%), median JAK2 V617F allele burden was 40% (IQR 17.8-66.2). Symptoms at diagnosis were present in 18/86 evaluable pts (20.9%), with pruritus accounting for 50% of cases (9/18). Arterial hypertension, diabetes, dyslipidemia and smoke attitude were present in 69.7%, 13.1%, 32.0% and 23.1% of pts, respectively. Previous thrombotic events were reported in 28/90 evaluable pts (38.1%). Hydroxyurea (HU) was started in 96/100 pts, after a median time of 1 month (IQR 0.1 - 3.8) from diagnosis. HU was discontinued in 20/96 pts (20.8%), mostly due to intolerance (12/20). Only 3/20 pts (15%) received Ruxolitinib as 2nd line, while no other drug was used in 11/20 pts (55%). Thrombotic events during follow up were reported in 10/86 evaluable pts (11.6%), while evolution in fibrotic and blastic phase was observed in 2 cases. At the last follow up, 18 pts died, 40 were lost to FU and 42 were still alive: 60-month and 120-month cumulative overall survival were 82.2% (95%CI 92.6 - 71.8) and 52.3% (95%CI 73.9 - 30.7), respectively Conclusion: Our data in a relatively large real-life cohort of very elderly PV pts highlight some points of the current clinical practice: as expected, few pts underwent marrow biopsy at diagnosis and quite all pts received HU as frontline cytoreductive therapy at a short interval after diagnosis. However, responsible physicians were still reluctant after HU discontinuation in prescribing 2nd line treatment with ruxolitinib in this setting. The high number of cases lost to follow-up, probably due to difficulties in reaching hematologic Center by these aged pts, made difficult a correct evaluation of OS curve, which however was similar to that of general population in the same age group

Breccia:AOP: Honoraria; BMS: Honoraria; Abbvie: Honoraria; Pfizer: Honoraria; Incyte: Honoraria; Novartis: Honoraria; GSK: Honoraria. Latagliata:Abbvie: Honoraria; BMS: Honoraria; Novartis: Consultancy, Honoraria.