Приобретенная апластическая анемия: особенности клинико-лабораторных проявлений и течения болезни, анализ лечения
DOI:
https://doi.org/10.15574/SP.2016.78.33Ключевые слова:
дети, приобретенная апластическая анемия, иммуносупрессивная терапия, трансплантация костного мoзгаАннотация
Представлен анализ клинико-лабораторных проявлений, особенностей течения приобретенной апластической анемии (ПАА) у детей, а также показана эффективность лечения 20 пациентов. Установлено, что наиболее эффективным методом лечения ПАА является аллогенная трансплантация костного мозга от семейного или неродственного донора, наименее эффективным терапевтическим подходом к лечению ПАА является посиндромная терапия. Программную иммуносупрессивную терапию (ИСТ) получили 14 больных. Часть пациентов во время первого курса ИСТ получили антитимоцитарный иммуноглобулин кролика, другие — антилимфоцитарный иммуноглобулин конский. При применении ИСТ первый ее курс был эффективным только у трети детей, треть детей умерли на разных этапах лечения от геморрагических и инфекционных осложнений; после второго курса ИСТ положительный ответ достигнут у 75,0% детей, у 14,3% детей, которым применялась ИСТ, возникли вторичные клональные заболевания, 7,1% детей оказались резистентными к лечению.
Ключевые слова: дети, приобретенная апластическая анемия, иммуносупрессивная терапия, трансплантация костного мoзга.
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