MPN-270 Challenges of the Management of Primary Myelofibrosis in Armenia.

Abstract

The aim of our work was to evaluate the diagnostic and therapeutic challenges and estimate survival for patients with primary myelofibrosis (PMF) in Armenia for the last decade. The data were collected from the medical records of all patients diagnosed with PMF in Armenia from 01 January 2010 to 31 December 2020. Survival analysis was performed using the Kaplan-Meier method. The total number of patients was 238, of whom 112 (47.1%) were alive at the time of data collection. The annual incidence was 0.93 cases per 100,000. The median age was 60 years (28-88 years), and 42.9% (102) of the patients were female. In all 112 patients, the diagnosis was based on the typical megakaryocyte changes accompanied by grade ≥2 reticulin/collagen fibrosis of bone marrow. The presence of JAK2, CALR, or MPL mutations or other clonal markers was estimated only in a limited number of patients: JAK2 in 10.7% (11) of patients, of which 6 were positive; MPL in 1 patient, which was positive; CALR and other clonal markers were not estimated in any patient. BCR/ABL was estimated in 12 patients, and all were negative. Of the total patients, 74.1% (83) received treatment with hydroxycarbamide, 5.3% (6) with ruxolitinib, and 6.3% (7) with interferon alfa-2; 8% (9) were under observation, and 6.3% (7) received unknown treatment. None of the patients underwent allogeneic hematopoietic stem cell transplantation, which is not available in Armenia. Diagnostic and treatment limitations are due to financial reasons, as the majority of expenses are out of pocket. The median survival of patients was 44 months (95%CI: 30-58 months), and 5-year overall survival was 40%. This is the first comprehensive analysis of PMF in Armenia. It showed significant diagnostic and therapeutic limitations, which led to inferior outcomes.