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

• Published in: Blood 2024-11, Vol.144 (Supplement 1), p.6675-6675
• Main Authors: Tatarelli, Caterina, Breccia, Massimo, Santopietro, Michelina, Paciaroni, Katia, Di Veroli, Ambra, Maurillo, Luca, Romano, Atelda, Andriani, Alessandro, Tafuri, Agostino, Latagliata, Roberto
• Format: Article
• Language: English
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood-2024-207330

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 cas