CLINICAL AND GENETIC FEATURES AND PROGNOSIS FACTORS FOR PREDICTING THE SURVIVAL OF PRIMARY MYELOFIBROSIS LITERATURE REVIEW.
Introduction: Primary myelofibrosis (PMF) is a common tumor from the group of Ph-negative myeloproliferative diseases (MPD). In the pathogenesis of MPD as a result of complex genetic rearrangements, the gene of the hematopoietic cell is damaged and its malignant transformation occurs. The review highlights recent data on the pathogenesis, clinical, morphological, and genetic characteristics of PMF. Particular attention is paid to diagnostic criteria, the principles of treating patients with the need for stratification to select management tactics, as well as determining the prognosis factors for their survival.
Purpose: A review of the literature on the diagnosis of primary myelofibrosis, stratification of patients for special adapted therapy depending on risk groups, as well as the determination of prognosis factors for survival in PMF.
Material and methods: Sources were searched in PubMed, Scopus, Ebscohost, Medline, The Сochrane Library, SpringerLink, Web of Knowledge (Thomson Reuters), Рaragraph Medicinе, Science Direct. Depth of search from 2013 to 2019. Inclusion criteria: reports on randomized and cohort studies conducted in large populations; meta-analyzes (9) and systematic reviews (5); Articles in English and Russian. Exclusion criteria: articles describing isolated cases and series of cases; materials published before 2013, materials that do not have evidence, summaries of reports, abstracts and newspaper articles. Of 114 literary sources, 33 articles were selected as analytical material for this article.
Results: The review focuses on diagnostic criteria, the principles of treatment of patients with PMF and the need for stratification to select management tactics, as well as determining prognosis factors for their survival.
Conclusions: Molecular genetic verification of primary myelofibrosis and stratification of patients using international unified systems contributes to the choice of therapeutic tactics algorithm and prediction of survival of patients with PMF.
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Baidurin S.A., Bekenova F.K., Ayapova M.Ye., Baranik T.F., Abdullina B.K., Dakhiyeva P.A., Afanasyeva A.A., Nakysh A.T., Кurmangaliyeva K.B. Clinical and genetic features and prognosis factors for predicting the survival of primary myelofibrosis. Review // Nauka i Zdravookhranenie [Science & Healthcare]. 2020, (Vol.22) 1, pp. 17-26. doi:10.34689/SH.2020.22.1.002Related publications:
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