Primary myelofibrosis: own experience and news from diagnostic and treatment
The newest advances in primary myelofibrosis (PMF) pathogenesis study, diagnostic and treatment approaches are presented in this article. The JAK-STAT signal pathway activation now recognized as main pathogenesis mechanism of PMF, it caused by JAK2, CALR, MPL genes mutations. Authors demonstrate the...
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| Format: | Article |
| Language: | Russian |
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ABV-press
2015-06-01
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| Series: | Онкогематология |
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| Online Access: | https://oncohematology.abvpress.ru/ongm/article/view/142 |
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| author | K. M. Abdulkadyrov V. A. Shuvaev I. S. Martynkevich |
| author_facet | K. M. Abdulkadyrov V. A. Shuvaev I. S. Martynkevich |
| author_sort | K. M. Abdulkadyrov |
| collection | DOAJ |
| description | The newest advances in primary myelofibrosis (PMF) pathogenesis study, diagnostic and treatment approaches are presented in this article. The JAK-STAT signal pathway activation now recognized as main pathogenesis mechanism of PMF, it caused by JAK2, CALR, MPL genes mutations. Authors demonstrate their own data about epidemiology, clinical signs, diagnostic and treatment results of 315 PMF patients. The most frequent clinical symptoms are: anemia, leukocytosis, thrombocytosis, splenomegaly, constitutional symptoms. Diagnostic criteria, prognostic scales (including cytogenetic and molecular features) issues are reviewed. Intermediate-1 risk grade is in the most proportion of patients. Therecommended PMF treatment algorithm is listed. The treatment methods, target drugs (Janus kinases inhibitors) trials results are discussed. |
| format | Article |
| id | doaj-art-fe388c7b6fcd4544aaa958d1adbe9dba |
| institution | DOAJ |
| issn | 1818-8346 2413-4023 |
| language | Russian |
| publishDate | 2015-06-01 |
| publisher | ABV-press |
| record_format | Article |
| series | Онкогематология |
| spelling | doaj-art-fe388c7b6fcd4544aaa958d1adbe9dba2025-08-20T03:21:30ZrusABV-pressОнкогематология1818-83462413-40232015-06-01102263610.17650/1818-8346-2015-10-2-26-36157Primary myelofibrosis: own experience and news from diagnostic and treatmentK. M. Abdulkadyrov0V. A. Shuvaev1I. S. Martynkevich2Russian Research Institute of Hematology and Transfusiology, Federal Medical and Biological Agency; 16, 2 nd Sovetskaya St., St. Petersburg, 191024, RussiaRussian Research Institute of Hematology and Transfusiology, Federal Medical and Biological Agency; 16, 2 nd Sovetskaya St., St. Petersburg, 191024, RussiaRussian Research Institute of Hematology and Transfusiology, Federal Medical and Biological Agency; 16, 2 nd Sovetskaya St., St. Petersburg, 191024, RussiaThe newest advances in primary myelofibrosis (PMF) pathogenesis study, diagnostic and treatment approaches are presented in this article. The JAK-STAT signal pathway activation now recognized as main pathogenesis mechanism of PMF, it caused by JAK2, CALR, MPL genes mutations. Authors demonstrate their own data about epidemiology, clinical signs, diagnostic and treatment results of 315 PMF patients. The most frequent clinical symptoms are: anemia, leukocytosis, thrombocytosis, splenomegaly, constitutional symptoms. Diagnostic criteria, prognostic scales (including cytogenetic and molecular features) issues are reviewed. Intermediate-1 risk grade is in the most proportion of patients. Therecommended PMF treatment algorithm is listed. The treatment methods, target drugs (Janus kinases inhibitors) trials results are discussed.https://oncohematology.abvpress.ru/ongm/article/view/142primary myelofibrosisjanus kinase gene mutationcalreticulin gene mutationprognostic scalestreatment algorithmruxolitinib |
| spellingShingle | K. M. Abdulkadyrov V. A. Shuvaev I. S. Martynkevich Primary myelofibrosis: own experience and news from diagnostic and treatment Онкогематология primary myelofibrosis janus kinase gene mutation calreticulin gene mutation prognostic scales treatment algorithm ruxolitinib |
| title | Primary myelofibrosis: own experience and news from diagnostic and treatment |
| title_full | Primary myelofibrosis: own experience and news from diagnostic and treatment |
| title_fullStr | Primary myelofibrosis: own experience and news from diagnostic and treatment |
| title_full_unstemmed | Primary myelofibrosis: own experience and news from diagnostic and treatment |
| title_short | Primary myelofibrosis: own experience and news from diagnostic and treatment |
| title_sort | primary myelofibrosis own experience and news from diagnostic and treatment |
| topic | primary myelofibrosis janus kinase gene mutation calreticulin gene mutation prognostic scales treatment algorithm ruxolitinib |
| url | https://oncohematology.abvpress.ru/ongm/article/view/142 |
| work_keys_str_mv | AT kmabdulkadyrov primarymyelofibrosisownexperienceandnewsfromdiagnosticandtreatment AT vashuvaev primarymyelofibrosisownexperienceandnewsfromdiagnosticandtreatment AT ismartynkevich primarymyelofibrosisownexperienceandnewsfromdiagnosticandtreatment |