Сімейний гемофагоцитарний лімфогістіоцитоз
ORPHA:540· ICD-10 D76.1· Familial hemophagocytic lymphohistiocytosis
Визначення(English summary)
Familial Hemophagocytic lymphohistiocytosis (FHL) is a rare primary immunodeficiency characterized by a macrophage activation syndrome (see this term) with an onset usually occurring within a few months or less common several years after birth.
- Поширеність
- Unknown
- Успадкування
- Autosomal recessive
- Вік початку
- Adolescent, Infancy