Болезнь Эрдгейма-Честера

Авторы: Доктор Джулио Кавалли, Доктор Алессандро Томерелли, Доктор Элвис Берти, Доктор Коррадо Кампочиаро, Доктор Лоренцо Дазна
Описание:

Болезнь Эрдгейма-Честера (ЭЧБ) является редким заболеванием, относится к нелангергансовым гистиоцитозам неизвестной этиологии, характеризуется ксантогранулематозной инфильтрацией различных тканей пенистыми гистиоцитами классов CD68+, CD1a-, S-100- (1,2).

Потенциально ЭЧБ может развиться в любом органе или ткани. Клиника зависит от длительности развития и распространенности патологических очагов, может варьировать от бессимптомного поражения костной ткани до мультисистемных форм, угрожающих жизни (3).

Диагностика этого заболевания затруднена, общий прогноз неблагоприятен. В последние годы применяются новые патогенетические подходы к лечению ЭЧБ с применением биологически активных веществ и ингибиторов BRAF. В статье мы обсудим основы ЭЧБ, начиная с этиопатогенеза, и заканчивая современными подходами к лечению.


Эпидемиология

ЭЧБ является весьма редким заболеванием, и зачастую упускается. С момента первого описания учеником Якоба Эрдгейма, Вильямом Честером в 1930 году (4), было выявлено более 500 случаев заболевания. В последние годы число вновь диагностируемых случаев существенно выросло из-за повышенного внимания медицинского сообщества.

ЭЧБ чаще встречается у мужчин (соотношение мужчины:женщины составляет 1,5), различия в клинике заболевания между полами отсутствуют (3). Наиболее часто болезнь поражает мужчин среднего возраста (медиана возраста пациентов с впервые поставленным диагнозом составляет 53 года)(3). ЭЧБ редко развивается у детей, описаны только несколько случаев (5,6).



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