Комбинированный иммунодефицит вследствие дефицита CD7
Определение
A rare autosomal recessive primary immunodeficiency characterized by susceptibility to Epstein-Barr virus (EBV)-related disorders (B-cell lymphoproliferative disorders including Hodgkin lymphoma) as well as dysgammaglobulinemia and recurrent infections. Patients can present with recurrent fever, lymphadenopathy, hepatosplenomegaly, Behþet-like stomatitis, pharyngitis, tonsillitis, adenitis, and viral encephalitis.