NEW APPROVED DRUG FOR THE TREATMENT OF ADULTS WITH POLYCYTHEMIA VERA

Arky J. Langtieh*, Julie B. Wahlang, Chayna Sarkar, Pavan B. Kalyan, Diana Nahakpam, Pratoy Baruah, Sabapathy H. Kumar

Polycythemia vera (PV), a myeloproliferative neoplasm (MPN), is a disease where there is an increased red cell mass. The pathology lies in the clonal proliferation in the erythroid lineage, granulocytic and megakaryocytic. Genetic mutation is seen in JAK2 phenotype where 95% of patients have V617F mutation.[1-3] The presenting symptoms range from pruritis, fatigue, headaches and progression of the disease leads to thrombosis, hemorrhage and acute myeloid leukemia. Blood counts present as increased erythrocytosis and increased hematocrit and hemoglobin concentrations. The mainstay of treatment for patients of all ages is phlebotomy and cytoreductive therapy which aims to bring down hematocrit value below 45%. The main drawback of these two therapies is failure to achieve the required hematocrit value and as such many patients suffer from iron deficiency anemia.[4-6] Rusfertide is a new drug whose novel mechanism of action is to mimic the action of endogenous hepcidin to control erythropoeisis and decreased hematocrit value below 45% in patients with Polycythemia Vera (PV).