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Izvestia Journal of the Union of Scientists - Varna. Medicine and Ecology Series

Severity of clinical manifestation, and prognosis of patients with myelofibrosis

Stela Dimitrova, Ilina Micheva


Introduction: Primary myelofibrosis (PMF) is a myeloproliferative disorder characterized by bone marrow fibrosis and ineffective extramedullary hematopoiesis, which presents with anemia, constitutional symptoms, and excessive splenomegaly. A number of factors are involved in the pathogenesis of the anemia in PMF, including impaired iron metabolism regulation. It was found that higher levels of the key regulator of iron metabolism - the peptide hormone hepcidin in patients with PMF, are associated with the severity of the anemia, blood transfusion dependence and decreased overall survival.

Aim: The aim of the study was to analyze the serum levels of hepcidin in patients with PMF and its impact on the clinical course, prognosis, and outcome of the disease.

Materials and Methods: A total of 68 patients with PMF and 12 healthy controls were analyzed. Serum hepcidin levels were measured by ELISA.  The results were statistically analyzed by dispersion, comparison, and correlation methods.

Results: Then mean hepcidin levels in patients with PMF were statistically significantly higher compared to healthy controls. (99.05 ng/mL; 20.57 ng/mL; F = 7.95; p = 0.006). High levels of hepcidin correlated with high risk according to DIPSS (p = 0.046), carrier of JakV617F mutation (p = 0.022), fibrotic phase according to WHO 2016 (p = 0.062), and the number of blood transfusions per month (p = 0.005). Higher hepcidin levels were not relevant to overall survival.

Conclusion: Hepcidin is a biological marker the monitoring of which in the course of MF would help for a more accurate clinical and prognostic assessment of the disease.


hepcidin, myelofibrosis, anemia, myeloproliferative diseases, iron metabolism

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