X-linked Severe Combined Immunodeficiency - Pathophysiology

Pathophysiology

X-SCID is characterized by an abnormally low number of lymphocytes and susceptibility to various infections. Susceptibility is in part due to the atrophy of the thymus, an organ required for T cell development, owing to absence of the receptors for IL-2, IL-7, and IL-15. These receptors are also essential for lymphocyte survival and homeostasis.