Առաջնային միելոֆիբրոզի ժամանակակից ախտորոշում և բուժում

<jats:p>Primary myelofibrosis (PMF) is a chronic hematologic disorder belonging to the group of Philadelphia chromosome–negative myeloproliferative neoplasms, characterized by clonal proliferation originating from hematopoietic stem cells. The disease is frequently, though not invariably, associated with mutations in the JAK2, CALR, and MPL genes.The pathogenesis of PMF is characterized by reticulin and/or collagen bone marrow fibrosis, aberrant expression of pro-inflammatory cytokines, hepatosplenomegaly, extramedullary hematopoiesis, anemia, constitutional symptoms, cachexia, and an increased risk of leukemic transformation. Diagnosis is based on histological examination of the bone marrow and molecular genetic testing. Modern treatment includes hydroxyurea, JAK inhibitors, and allogeneic hematopoietic stem cell transplantation (AHSCT).</jats:p>