Множественная миелома (патогенез, клиника, диагностика, дифференциальный диагноз). Часть I

С.С. Бессмельцев

Российский научно-исследовательский институт гематологии и трансфузиологии, Санкт-Петербург, Российская Федерация


РЕФЕРАТ

Множественная миелома (ММ) — опухолевое заболевание, характеризующееся инфильтрацией костного мозга плазматическими клетками и обширным поражением костей скелета, что сопровождается болью и переломами костей. В обзоре представлены лабораторные признаки и клинические симптомы, характерные для ММ. Диагноз ММ не вызывает сомнений при обнаружении в костном мозге больных 10 % и более клональных плазматических клеток, M-протеина в сыворотке или моче (кроме несекретирующей миеломы), гиперкальциемии, почечной недостаточности, анемии или очагов лизиса в костях скелета. Для выявления М-протеина используют электрофорез сывороточных белков и иммунофиксацию. Кроме того, необходимы электрофорез и иммунофиксация мочевых белков или определение свободных легких цепей иммуноглобулинов. Международная система стадирования в зависимости от уровня b2-микроглобулина и альбумина в сыворотке подразделяет больных ММ на три группы риска: стандартного, промежуточного и высокого. Анализ хромосомных нарушений позволяет более надежно стратифицировать больных на группы риска. Установлено, что del(13), t(4;14) или t(14;16), del(17p), гиподиплоидия и индекс метки плазматических клеток 3 % и более существенно ухудшают прогноз ММ.

В обзоре представлены лабораторные признаки и клинические симптомы моноклональной гаммапатии неясного генеза/неопределенного значения, асимптоматической миеломы (тлеющей миеломы), несекретирующей миеломы, солитарной плазмоцитомы кости, экстрамедуллярной плазмоцитомы, плазмоклеточного лейкоза, макроглобулинемии Вальденстрема, амилоидоза и других заболеваний.


Ключевые слова: множественная миелома, моноклональные гаммапатии неясного генеза, асимптоматическая миелома, M-протеин, клональные плазматические клетки.

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