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Отсюда и название: «миелос» означает костный мозг, а «фиброз» — развитие фиброзной или рубцовой ткани [1](https://rare-aid.com/where_to_turn/rare_disease/mielofibroz/). \n\n**Распространённость**: от 0,3 до 0,7 случаев на 100 тыс. населения [5](https://www.KrasotaiMedicina.ru/diseases/hematologic/myelofibrosis). Пик заболеваемости — возраст от 50 до 70 лет, но встречаются и молодые пациенты [5](https://www.KrasotaiMedicina.ru/diseases/hematologic/myelofibrosis). Чаще страдают мужчины [5](https://www.KrasotaiMedicina.ru/diseases/hematologic/myelofibrosis). \n\n\n\n## Причины\n**Точная причина первичного миелофиброза** не установлена [5](https://www.KrasotaiMedicina.ru/diseases/hematologic/myelofibrosis). Некоторые факторы, которые могут увеличивать риск: \n* генетические мутации (например, в гене JAK2) [1](https://rare-aid.com/where_to_turn/rare_disease/mielofibroz/)[6](https://navigator.mosgorzdrav.ru/diseases/onkologiya/mielofibroz/); \n* воздействие определённых химических веществ (бензола, толуола) [6](https://navigator.mosgorzdrav.ru/diseases/onkologiya/mielofibroz/)[8](https://navigator.mosgorzdrav.ru/diseases/onkologiya/mielofibroz/?sphrase_id=2162386); \n* воздействие радиации [6](https://navigator.mosgorzdrav.ru/diseases/onkologiya/mielofibroz/)[8](https://navigator.mosgorzdrav.ru/diseases/onkologiya/mielofibroz/?sphrase_id=2162386). \n\n**Вторичный миелофиброз** развивается как исход других хронических миелопролиферативных заболеваний — истинной полицитемии, эссенциальной тромбоцитемии, хронического миелолейкоза [5](https://www.KrasotaiMedicina.ru/diseases/hematologic/myelofibrosis). \n\n## Симптомы\n**На ранних стадиях** у многих больных заболевание протекает бессимптомно [9](https://www.humanitashospitals.ru/bolezni/mielofibroz/). **По мере прогрессирования** возможны: \n* усталость, слабость или затруднение дыхания из-за анемии [9](https://www.humanitashospitals.ru/bolezni/mielofibroz/); \n* боль или чувство полноты в левом подреберье из-за увеличения селезёнки [9](https://www.humanitashospitals.ru/bolezni/mielofibroz/); \n* бледная кожа [9](https://www.humanitashospitals.ru/bolezni/mielofibroz/); \n* частое появление синяков [9](https://www.humanitashospitals.ru/bolezni/mielofibroz/); \n* склонность к кровотечениям [9](https://www.humanitashospitals.ru/bolezni/mielofibroz/); \n* чрезмерная потливость во время сна (ночной пот) [9](https://www.humanitashospitals.ru/bolezni/mielofibroz/); \n* повышение температуры [9](https://www.humanitashospitals.ru/bolezni/mielofibroz/); \n* частые инфекции [9](https://www.humanitashospitals.ru/bolezni/mielofibroz/); \n* боль в костях [9](https://www.humanitashospitals.ru/bolezni/mielofibroz/). \n\n## Диагностика\n**Для диагностики миелофиброза проводят**: \n* **Анализы крови** — общий анализ показывает аномально низкий уровень эритроцитов (признак анемии) [6](https://navigator.mosgorzdrav.ru/diseases/onkologiya/mielofibroz/)[8](https://navigator.mosgorzdrav.ru/diseases/onkologiya/mielofibroz/?sphrase_id=2162386). \n* **Биопсию костного мозга** — с её помощью определяют количество незрелых клеток в костном мозге [3](https://www.kp.ru/doctor/bolezni/pervichnyj-mielofibroz/). \n* **Генетический анализ** — образец крови или костного мозга анализируют на генные мутации, связанные с миелофиброзом [6](https://navigator.mosgorzdrav.ru/diseases/onkologiya/mielofibroz/)[8](https://navigator.mosgorzdrav.ru/diseases/onkologiya/mielofibroz/?sphrase_id=2162386). \n\n## Лечение\n**Основная цель лечения** — облегчить симптомы и уменьшить риск осложнений [1](https://rare-aid.com/where_to_turn/rare_disease/mielofibroz/). **Некоторые методы**: \n* **Медикаментозная терапия** — глюкокортикостероидные препараты, средства, стимулирующие эритропоэз, препараты интерферона-альфа [2](https://yandex.ru/medicine/disease/mielofibroz). \n* **Переливание компонентов крови** — при выраженной анемии [1](https://rare-aid.com/where_to_turn/rare_disease/mielofibroz/)[2](https://yandex.ru/medicine/disease/mielofibroz). \n* **Хирургическое лечение** — спленэктомия (операция по удалению селезёнки) показана, если другие методы лечения не помогли уменьшить её размер [1](https://rare-aid.com/where_to_turn/rare_disease/mielofibroz/). \n* **Трансплантация стволовых клеток** — проводят молодым пациентам без сопутствующих болезней при наличии подходящего донорского материала [1](https://rare-aid.com/where_to_turn/rare_disease/mielofibroz/). \n\n**В некоторых случаях**, когда заболевание протекает в лёгкой форме, без признаков анемии, увеличения селезёнки или других осложнений, лечение обычно не требуется — показаны регулярные осмотры и обследования для наблюдения за прогрессированием болезни [1](https://rare-aid.com/where_to_turn/rare_disease/mielofibroz/)[11](https://navigator.mosgorzdrav.ru/diseases/onkologiya/mielofibroz/?sphrase_id=2112774). 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