Baidurin S, Bekenova F, Rakhimbekova G, Abdullina B, Nakysh A
NJSC "Astana Medical University", Department of Internal Medicine with a course of nephrology, hematology, allergology and immunology.
Georgian Med News. 2020 Dec(309):86-94.
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.
原发性骨髓纤维化是一种常见于Ph阴性骨髓增殖性疾病的病症。本文介绍了关于Ph阴性骨髓增殖性疾病发病机制的现代数据，以及原发性骨髓纤维化的诊断标准、治疗策略和预后因素。文中描述了一例原发性骨髓纤维化转化为急性髓系白血病的临床病例。本研究的目的是提供有关原发性骨髓纤维化发病机制、诊断标准、治疗原则和预后因素的最新信息，并呈现原发性骨髓纤维化转化为急性髓系白血病的临床病例。根据现代观念，对于原发性骨髓纤维化的早期诊断，除了对患者进行临床和形态学检查方法外，对该疾病进行分子遗传学验证极为重要。为提高原发性骨髓纤维化患者的生存率，对该疾病进行分子遗传学验证并进行分层以选择治疗策略是必要的。
