Клинико-гематологическая и биологическая вариабельность миелодиспластического синдрома и смешанных миелоидных заболеваний
Несомненным достижением классификации ВОЗ опухолей кроветворной и лимфоидной тканей является создание новой, четвертой, группы миелоидных заболеваний (МДС/МПЗ) и выделение в самостоятельную нозологическую единицу вторичных МДС и OMJI. В тоже время в классификации не отражено многообразие клинических вариантов МДС/МПЗ. Неопределенность позиции смешанных миелоидных заболеваний с пролиферацией… Читать ещё >
Содержание
- Глава 1. Обзор литературы 17 Миелодиспластический синдром и смешанные миелоидные заболевания: классификация, патогенез и терапия низкой интенсивности
- 1. 1. Определение МДС
- 1. 2. Классификации МДС
- 1. 2. 1. FAB-классификация
- 1. 2. 2. Классификация ВОЗ опухолей кроветворной и лимфоидной 19 тканей
- 1. 2. 3. Международная шкала прогноза IPSS
- 1. 3. Ген опухоли Вилмса и его прогностический потенциал у 27 больных МДС
- 1. 4. Роль иммунных механизмов в патогенезе МДС
- 1. 5. Иммуносупрессивная терапия больных МДС
- 1. 6. Терапия рекомбинантным эритропоэтином больных МДС
- 1. 7. Антицитокиновая терапия больных МДС
- 1. 8. Вариабельность смешанных МДС/МПЗ
- 1. 9. Вторичный МДС у больных миеломой
- Глава 2. Объект, материалы и методы исследования
- 2. 1. Объект и материалы исследования
- 2. 2. Диагностика МДС, определение группы риска и критерии 57 гематологического ответа на терапию низкой интенсивности
- 2. 3. Диагностика МДС/МПЗ
- 2. 4. Методы терапии низкой интенсивности больных МДС
- 2. 5. Лабораторные методы исследования
- 2. 6. Методы статистической обработки
- Глава 3. Прогностический потенциал морфологических и цитогенетических показателей у больных de novo МДС
- 3. 1. Клинико-гематологическая характеристика больных
- 3. 2. Частота лейкозной трансформации у больных МДС
- 3. 3. Трансформация в OMJI в группах больных МДС с разным 66 содержанием бластов в костном мозге
- 3. 4. Трансформация в OMJI в группах больных МДС с разным 68 кариотипом
- 3. 5. Трансформация в OMJI в группах больных МДС с разным IPSS 69 прогнозом
- 3. 6. Результаты одно- и многофакторного анализов
- Глава 4. Экспрессия гена опухоли Вилмса в лейкоцитах крови больных 71 МДС
- 4. 1. Клинико-гематологическая характеристика больных
- 4. 2. Частота экспрессии WT1 в лейкоцитах крови больных МДС
- 4. 3. Течение de novo МДС у больных с экспрессией WT
- 4. 4. Течение de novo МДС у больных без экспрессии WT
- 4. 5. Сравнительная характеристика клинико-гематологических 76 показателей и течения de novo МДС у больных с экспрессией и без экспрессии WT
- 4. 6. Статистический анализ данных обследования больных de novo 77 МДС
- Глава 5. Субпопуляционный состав лимфоцитов крови и продукция 78 ФНО-а клетками крови больных первичным МДС
- 5. 1. Субпопуляционный состав лимфоцитов крови
- 5. 2. Спонтанная продукция ФНО-а клетками крови
- 5. 3. Корреляции между содержанием лимфоцитов в крови и 83 спонтанной продукцией ФНО-а клетками крови
- Глава 6. Иммуносупрессивная терапия больных МДС
- 6. 1. Клинико-гематологическая характеристика больных
- 6. 2. Эффективность ИСТ
- 6. 3. Ответ на ИСТ
- 6. 4. Сроки наступления и длительность ответа на ИСТ
- 6. 5. Отмена ИСТ в ранние сроки
- 6. 6. Токсические осложнения, развившиеся на ИСТ
- Глава 7. Терапия больных МДС рекомбинантным эритропоэтином
- 7. 1. Клинико-гематологическая характеристика больных
- 7. 2. Результаты терапии
- Глава 8. Антицитокиновая терапия больных МДС
- 8. 1. Клинико-гематологическая характеристика больных
- 8. 2. Результаты терапии
- Глава 9. Клинико-биологическая вариабельность смешанных 110 миелоидных заболеваний
- 9. 1. МДС/МПЗ с нейтрофилезом
- 9. 2. МД С/МПЗ с эозинофилией
- 9. 3. МДС/МПЗ с тромбоцитозом
- 9. 4. МДС/МПЗ с базофилией
- Глава 10. Вторичный МДС у больных множественной миеломой
- 10. 1. Клинико-гематологическая характеристика больных
- 10. 2. Вариабельность вторичного МДС
- Заключение
- Выводы 150 Практические рекомендации
- Список литературы
СПИСОК СОКРАЩЕНИЙ АА — апластическая анемия Ара-С — арабинозид АЛГ — антилимфоцитарный глобулин АТГ — антитимоцитарный глобулин АЧН — абсолютное число нейтрофилов ГО — гематологический ответ
Г-КСФ — гранулоцитарный колониестимулирующий фактор ГМ-КСФ — гранулоцитарно-макрофагальный колониестимулирующий фактор
ГУ — гематологическое улучшение
ГУ-Н (БО) — большой нейтрофильный ответ
ГУ-Т (БО) — большой тромбоцитарный ответ
ГУ-Т (МО) — малый тромбоцитарный ответ
ГУ-Э (БО) — большой эритроцитарный ответ
ГУ-Э (МО) — малый эритроцитарный ответ
Декса — дексаметазон
З.Н. — зрелые нейтрофилы
Ида — идарубицин
ИНФ — интерферон
ИСТ — иммуносупрессивная терапия
КМ — костный мозг
КОЕ — колониеобразующая единица
КОЕ-ГМ — колониеобразующие клетки грануломоноцитопоэза
КС — кортикостероиды
ЛДГ — лактатдегидрогеназа
МДС — миелодиспластический синдром
МДС/МПЗ — миелодиспластический синдром/миелопролиферативное заболевание
М-КСФ — макрофагальный колониестимулирующий фактор
МКЦ — миелокариоциты
ММ — множественная миелома
ММФ — микофенолат мофетил
МНК — мононуклеарные клетки
6-МП — 6-меркаптопурин
МПЗ — миелопролиферативное заболевание
М.Р. — мегакариоцитарный росток
Н.Н. — незрелые нейтрофилы
OJI — острый лейкоз
OMJT — острый миелобластный лейкоз pOMJI — резистентный острый миелобластный лейкоз
ПлК — плазматические клетки
ПК — периферическая кровь
ПР — полная ремиссия
ПТ- поддерживающая терапия
ПТК — пентоксифиллин
РА — рефрактерная анемия
РАИБ — рефрактерная анемия с избытком бластов
РАИБ-Т — рефрактерная анемия с избытком бластов в трансформации
РАКС — рефрактерная анемия с кольцевыми сидеробластами
РТ — рентгентерапия
МД — рефрактерная цитопения с мультилинейной диспазией
ТГСК — трансплантация стволовых клеток аХМЛ — атипичный хронический миелолейкоз
XMMoJT — хронический миеломоноцитарный лейкоз юММоЛ — ювенильный миеломоноцитарный лейкоз
ХГЛ — хронический гранулоцитарный лейкоз
XT — химиотерапия
ФНО-а — фактор некроза опухоли альфа Ципро — ципрофлоксацин
ЦсА — циклоспорин А
ЩФ — щелочная фосфатаза нейтрофилов
ЭО — эритроцитарный ответ рЭпо — рекомбинантный эритропоэтин эЭпо — эндогенный эритропоэтин
Эр.Р.- эритроидный росток
BFU-E — колониеобразующие единицы эритропоэза
Р2М — бета-2 микроглобулин
Complex — комплексные повреждения кариотипа
CFU-Es — колониеобразуюшие единицы эритропоэза
CMV — цитомегаловирус
HSV — вирус простого герпеса
IPSS — Международная шкала прогноза
IWG — Международная рабочая группа
МНС — главный комплекс гистосовместимости
PCD — пентоксифиллин, ципрофлоксацин, дексаметазон
Ph' - филадельфийская хромосома
WT1 — ген опухоли Вилмса
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