ADVANCED THERAPY IN PATIENTS WITH PRIMARY AND POSTPOLYCYTHEMIC MYELOFIBROSIS
Rubrics: HEMATOLOGY
Abstract and keywords
Abstract (English):
Aktual'nost' problemy obuslovlena nizkoy effektivnost'yu lecheniya i plohim prognozom mielofibroza do poslednego vremeni. Mielofibroz yavlyaetsya ugrozhayuschim zhizni zlokachestvennym novoobrazovaniem krovi s neblagopriyatnym prognozom i ogranichennym kolichestvom variantov lecheniya. Po rezul'tatam issledovaniy u bol'nyh mielofibrozom nablyudaetsya umen'shenie ozhidaemoy prodolzhitel'nosti zhizni s medianoy 5,7 let. Hotya allogennaya transplantaciya stvolovyh kletok mozhet privesti k izlecheniyu mielofibroza, dannaya procedura soprovozhdaetsya znachitel'noy chastotoy oslozhneniy i svyazannoy s ney smertnost'yu, takim obrazom, ee provedenie vozmozhno menee chem u 5 % bol'nyh molodogo vozrasta s horoshim sostoyaniem zdorov'ya. Poyavlenie novogo effektivnogo i bezopasnogo preparata dlya lecheniya mielofibroza Dzhakavi pozvolit dobit'sya luchshego kachestva zhizni i snizheniya simptomov u pacientov s dannoy patologiey. Cel'yu nashego issledovaniya stala ocenka effektivnosti ruksolitiniba u pacientov s diagnozami pervichnyy mielofibroz i post-policitemicheskiy mielofibroz, nahodivshihsya na lechenii v gematologicheskom otdelenii RKB im. N.A. Semashko. Poluchennye rezul'taty sootvetstvuyut dannym, poluchennym na bol'shih gruppah bol'nyh v issledovaniyah 3-y fazy COMFORT-I i COMFORT-II.

Keywords:
pervichnyy mielofibroz, mutaciya-JAK2, hMPZ, targetnaya terapiya
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