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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Main Authors: K. M. Abdulkadyrov, V. A. Shuvaev, I. S. Martynkevich
Format: Article
Language:Russian
Published: ABV-press 2015-06-01
Series:Онкогематология
Subjects:
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.
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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