Functional T cell immunodeficiencies (with T cells present)
LD Notarangelo - Annual review of immunology, 2013 - annualreviews.org
Severe combined immunodeficiency (SCID) comprises a group of disorders that are fatal
owing to genetic defects that abrogate T cell development. Numerous related defects have
recently been identified that allow T cell development but that compromise T cell function by
affecting proximal or distal steps in intracellular signaling. These functional T cell
immunodeficiencies are characterized by immune dysregulation and increased risk of
malignancies, in addition to infections. The study of patients with these rare conditions, and …
owing to genetic defects that abrogate T cell development. Numerous related defects have
recently been identified that allow T cell development but that compromise T cell function by
affecting proximal or distal steps in intracellular signaling. These functional T cell
immunodeficiencies are characterized by immune dysregulation and increased risk of
malignancies, in addition to infections. The study of patients with these rare conditions, and …