ABSTRACT
Primary myelofibrosis is a common disease from the group of Ph-negative myeloproliferative diseases. The article presents modern data on the pathogenesis of Ph-negative myeloproliferative diseases, as well as diagnostic criteria, treatment tactics and prognosis factors for primary myelofibrosis. A clinical case of transformation of primary myelofibrosis into acute myeloid leukemia is described. Purpose of the study - to present up-to-date information on the pathogenesis, diagnostic criteria, principles of treatment and prognostic factors of primary myelofibrosis, as well as to present a clinical case of transformation of primary myelofibrosis into acute myeloblastic leukemia. According to modern concepts, for the early diagnosis of primary myelofibrosis, along with the clinical and morphological methods of examining patients, molecular genetic verification of the disease is extremely important. To improve the survival rate of patients with primary myelofibrosis, molecular genetic verification of the disease and stratification for the choice of treatment tactics are necessary.
