Журнал «Практическая онкология» Том 5, №1, 2022

Мієлопроліферативні захворювання (МПЗ), що включають істинну поліцитемію (ІП), есенціальну тромбоцитемію (ЕT) і первинний мієлофіброз (ПМФ), є клональними порушеннями, ускладненими в основному судинними проявами і трансформацією в мієлофіброз (для ІП і ET) або лейкоз. Вторинні злоякісні новоутворення, зокрема лімфопроліферативні захворювання (ЛПЗ), зустрічаються значно рідше, однак вони виникають з більшою частотою, ніж у загальній популяції. В даному огляді ми зосередились на трьох питаннях: 1) роль JAK2 і шляхи JAK/STAT у виникненні МПЗ і ЛПЗ; 2) значення генетичної схильності у виникненні як МПЗ, так і ЛПЗ; 3) яке місце займають циторедуктивні препарати у виникненні МПЗ і ЛПЗ.

Myeloproliferative neoplasms (MPN), which include polycythemia vera, essential thrombocythemia, and primary myelofibrosis, are clonal disorders complicated mainly by vascular events and transformation to myelofibrosis (for polycythemia vera and essential thrombocythemia) or leukemia. Secondary malignancies, particularly lymphoproliferative disorders (LPN), are rare. They occur at a higher frequency than found in the general population. This review focused on three issues: 1) the role of JAK2 and JAK/STAT pathways in the emergence of MPN and LPN; 2) the importance of genetic predisposition in the occurrence of both MPN and LPN; 3) the place of cytoreductive drugs in causing the MPN and LPN.

інгібітори JAK; шлях JAK/STAT; JAK2; лімфопроліферативні захворювання; мієлопроліферативні захворювання; руксолітиніб

JAK inhibitors; JAK/STAT pathway; JAK2; lymphoproliferative disorders; myeloproliferative neoplasms; ruxolitinib

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