Polycythemia Vera in Clinical Practice: A Single-Center Experience Focusing on Thrombotic Complications and Management Strategies
摘要
Background: Polycythemia vera (PV) is a Philadelphia-negative myeloproliferative neoplasm characterized by erythrocytosis, where thrombosis is the major cause of morbidity and mortality, and hydroxyurea (HU) resistance or intolerance complicates management. This study evaluated clinical features, treatment response, thrombotic events, and survival in PV patients. Methods: We retrospectively analyzed 195 PV patients diagnosed between 1990 and 2021, confirmed according to the 2016 World Health Organisation criteria. Demographics, comorbidities, laboratory parameters, treatment responses, thrombotic complications, and survival outcomes were collected. HU resistance and intolerance were defined according to modified European Leukemia Net criteria. Results: The cohort included 124 males (63.6%) with a mean age of 56.6±12.8 years. Complete and partial responses to HU were achieved in 71.2% and 17.5% of patients, respectively. Thrombosis occurred in 29 patients (14.8%), including 62% arterial and 38% venous events. Patients with thrombotic events had higher rates of HU resistance (20.7%) and intolerance (34.4%), and elevated lactate dehydrogenase (LDH) levels at diagnosis and during thrombosis. The estimated mean OS was 252.5 ± 14.5 months, with 5- and 10-year survival rates of 97.1% and 85.1%, respectively. Survival did not differ significantly between patients with and without thrombosis. High Charlson Comorbidity Index scores were independently associated with poorer survival (HR: 13.22, p=0.014).Conclusion: Thrombosis is frequent in PV, but survival can be preserved with appropriate management. HU resistance and intolerance, elevated LDH, and high comorbidity burden are important predictors of adverse outcomes. These findings support individualized risk-adapted therapy and vigilant long-term follow-up to improve prognosis.