Objective: BCR/ABL-negative myeloproliferative neoplasms are characterized by over-production myeloid lineages in the bone marrow. Polycythemia vera, essential thrombocythemia and primary myelofibrosis are the most common myeloproliferative neoplasms. Diagnosis is made according to the WHO diagnostic criteria from clinical data, hematological and biochemical analysis and BM histology. The aim of this study was to analyse patient demographic characteristics, clinical features, laboratory findings, mutational status together with complications, clinical course and survival.
Methodology: This study was conducted on patients diagnosed with myeloproliferative neoplasms between 2008 and 2019. Hemogram and biochemical parameters, demographic information, mutation analysis, management, complications and follow-up periods were recorded for all patients. Survival rates were calculated and the effect of the parameters on overall survival was analyzed.
Results: Evaluation was made of 247 patients, comprising 105 polycythemia vera, 126 essential thrombocythemia and 16 primary myelofibrosis patients. The overall frequency of driver mutations was 96.1% for PV, 71.4% for ET and 75% for PMF. Hydroxyurea was the most commonly used first-line treatment agent and the most common indication for switching to second-line treatment in all disease subgroups was the development of side-effects. During follow-up, 11 polycythemia vera, 14 essential thrombocythemia and 2 primary myelofibrosis patients developed thromboembolic complications. Median overall survival could not be reached in polycythemia vera and essential thrombocythemia patient and determined as 70.3 months in primary myelofibrosis patients. Age, LDH, ferritin and platelet/lymphocyte ratio at the time of diagnosis and thromboembolic complications were determined to have a statistically significant effect on survival in all patients.
Conclusion: Lower survival rates were seen in the primary myelofibrosis patients although thromboembolic complications were observed at similar rates in all 3 disease subgroups. In addition to known risk factors such as age and thromboembolic complications, parameters such as LDH, ferritin and PLR, which may be considered to indicate disease activity and inflammation, can also be used as prognostic markers.
