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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