Retrospective evaluation of clinical, demographic, molecular data of BCR-ABL negative chronic myeloproliferative neoplasia

Objective: BCR-ABL negative myeloproliferative neoplasms are characterized by over-production of myeloid lineages in bone marrow. The most common forms are Polycythemia Vera (PV), Essential Thrombocytosis (ET) and Primary Myelofibrosis (PMF). We aimed to retrospectively evaluate the clinical, demographic and molecular data and survival analysis of these three groups of patients followed in Dokuz Eylul University Hematology Outpatient Clinic. Materials and methods: Data of 363 patients, including 230 patients with ET, 104 patients with PV, and 29 patients with PMF, were retrospectively analyzed in a single-center study, which was diagnosed according to WHO(World Healt Organization) 2016 criteria, between January 2012 and May 2021. Results: JAK-2 mutation was positive in 85.1% of patients with PV, 65.4% of patients with ET and 60% of patients with PMF. At diagnosis, thrombosis was detected in 18% of patients with PV, 19.1% of patients with ET, and 10.3% of patients with PMF. Three hundred and twenty patients (88.2%) received cytoreductive therapy. The most commonly used drug was hydroxyurea (HU) (85.7%). The rates of leukemic and fibrotic transformation were 1.6% and 14.5%. Age (p<0.001) and JAK2V617F (p=0.002) were found to be significant, two significant risk factors for thrombosis. The 10-year calculated overall survival was 90.3% in PV, 86% in ET, and 58.6% in PMF patients. In the multiple regression analysis on survival, a significant effect of age, presence of anemia, history of thrombosis and mutation analysis difference, which are prognostic indicators, were determined. Conclusion: Age and presence of JAK2 V617 F mutation are important risk factors for thromboembolic processes. In addition, subtype analyzes of CALR and MPL mutations are important factors in the prognostic evaluation. The most commonly used cytoreductive therapy to prevent complications is hydroxyurea, which still holds its place today. Keywords: Chronic myeloproliferative diseases, thrombosis, survival