OncohematologyOncohematologyОнкогематология1818-83462413-4023Publishing House ABV Press15110.17650/1818-8346-2015-10-3-28-42HEMATOLOGIC MALIGNANCIES: DIAGNOSIS, TREATMENT, SUPPORTIVE CAREГЕМОБЛАСТОЗЫ: ДИАГНОСТИКА, ЛЕЧЕНИЕ, СОПРОВОДИТЕЛЬНАЯ ТЕРАПИЯAll we know about polycythemia vera: literature review and own experienceЧто нам известно об истинной полицитемии (обзор литературы и собственные данные)AbdulkadyrovK. M.АбдулкадыровК. М.
Russian Federation

16, 2nd Sovetskaya St., St. Petersburg, 191024

191024, Санкт-Петербург, ул. 2-я Советская, 16

ShuvaevV. A.ШуваевВасилий Анатольевич
Russian Federation

16, 2nd Sovetskaya St., St. Petersburg, 191024

191024, Санкт-Петербург, ул. 2-я Советская, 16

shuvaev77@mail.ruMartynkevichI. S.МартынкевичИ. С.
Russian Federation

16, 2nd Sovetskaya St., St. Petersburg, 191024

191024, Санкт-Петербург, ул. 2-я Советская, 16

Russian Research Institute of Hematology and Transfusiology, Federal Medical and Biological AgencyФГБУ «Российский научно-исследовательский институт гематологии и трансфузиологии Федерального медико-биологического агентства»0810201510328420710201507102015https://oncohematology.abvpress.ru/ongm/article/view/151

The literature review and own long-term polycythemia vera diagnosis and treatment experience are presented in this article. The results of newest advances in pathogenesis description, modern diagnostic techniques and treatment modalities in polycythemia vera are included. The JAK-STAT signal pathway activation now recognized as main pathogenesis mechanism of polycythemia vera. In this case this activation caused almost exlusively by JAK2 gene mutations. Authors demonstrate their own data about epidemiology, clinical signs and diagnostic and treatment results of 252 polycythemia vera patients. The most frequent clinical symptoms at diagnosis were: plethora, headache and dizziness, fatigue, pruritus. Diagnostic criteria and thrombotic complications prognostic scale are presented. The thrombosis frequency in this polycythemia vera patients group was 11.1 %. It was included 3.6 % of myocardial infarctions and 5.2 % of strokes. The thrombotic complications rates statistically differed in various prognostic groups. For example, from 2.6 % in low-risk group to 20.6 % in high-risk thrombosis group. The used personalized polycythemia vera management algorithm is listed. The treatment methods features, target drugs (Janus kinases inhibitors) trials results are discussed.

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