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Імунодефіцит у дорослих з аутоантитілами до антиінтерферону-гамма

ORPHA:306431· ICD-10 D84.8· Adult-onset immunodeficiency with anti-interferon-gamma autoantibodies

Визначення(English summary)

A rare acquired immunodeficiency disorder characterized by the appearance of susceptibility to disseminated opportunistic infections (in particular, disseminated nontuberculous mycobacterial infection, salmonellosis, penicillosis, and varicella zoster virus infection) in previously healthy (HIV-negative) adults, associated with the presence of acquired autoantibodies to interferon gamma. Typical clinical manifestation includes lymphadenopathy (cervical or generalized), fever, weight loss and/or reactive skin lesions.

Поширеність
Unknown
Успадкування
Not applicable
Вік початку
Adult