X-LINKED IMMUNODEFICIENCY

Course overview

PRIMARY IMMUNODEFICIENCIES (PID) ARE A HETEROGENEOUS GROUP OF DISEASES, MOSTLY INHERITED, CAUSED BY MORE THAN 400 INTRINSIC DISORDERS OF THE IMMUNE SYSTEM. SEVEN DIFFERENT IMMUNE DEFICIENCY STATES, WHICH ARE TRANSMITTED WITH THE X CHROMOSOME, HAVE BEEN DESCRIBED. THOSE ARE X-LINKED CHRONIC GRANULOMATOUS DISEASE (X-CGD), X-LINKED IMMUNODEFICIENCY WITH HYPER-IGM, X-LINKED LYMPHOPROLIFERATIVE SYNDROME, WISKOTT-ALDRICH SYNDROME, X-LINKED SEVERE COMBINED IMMUNODEFICIENCY (X-SCID), X-LINKED AGAMMAGLOBULINEMIA (XLA), AND IPEX. THIS ACTIVITY REVIEWS THE EVALUATION AND TREATMENT OF X-LINKED IMMUNODEFICIENCY AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN EVALUATING AND TREATING PATIENTS WITH THIS CONDITION.