IMMUNODEFICIENCY

Course overview

IMMUNODEFICIENCY RESULTS FROM A FAILURE OR ABSENCE OF ELEMENTS OF THE IMMUNE SYSTEM, INCLUDING LYMPHOCYTES, PHAGOCYTES, AND THE COMPLEMENT SYSTEM. IMMUNODEFICIENCIES CAN BE PRIMARY OR SECONDARY. PRIMARY IMMUNODEFICIENCY IS SUBDIVIDED INTO TYPES THAT CAUSE T-CELL DEFICIENCY, B-CELL DEFICIENCY, BOTH T-CELL AND B-CELL DEFICIENCY, COMPLEMENT DEFICIENCY, PHAGOCYTE DEFICIENCY, AND IMMUNOGLOBULIN A DEFICIENCY. PRIMARY IMMUNODEFICIENCIES LEADING TO T-CELL DEFICIENCY INCLUDE DIGEORGE SYNDROME, ALSO KNOWN AS CONGENITAL THYMIC APLASIA, CHRONIC MUCOCUTANEOUS CANDIDIASIS, HYPER-IMMUNOGLOBULIN M SYNDROME, AND INTERLEUKIN-12 RECEPTOR DEFICIENCY. PRIMARY IMMUNODEFICIENCY LEADING TO B-CELL DEFICIENCY INCLUDES X-LINKED AGAMMAGLOBULINEMIA, ALSO CALLED BRUTON AGAMMAGLOBULINEMIA. PRIMARY IMMUNODEFICIENCIES LEADING TO BOTH T-CELL AND B-CELL DEFICIENCY INCLUDE SEVERE COMBINED IMMUNODEFICIENCY DISEASE, WISKOTT-ALDRICH SYNDROME, IMMUNODEFICIENCY WITH ATAXIA-TELANGIECTASIA, AND MAJOR HISTOCOMPATIBILITY COMPLEX DEFICIENCY. PRIMARY IMMUNODEFICIENCIES LEADING TO COMPLEMENT DEFICIENCY INCLUDE HEREDITARY ANGIOEDEMA, DEFICIENCY OF C3, DEFICIENCY OF MEMBRANE ATTACK COMPLEX, AND C2 OR C4 DEFICIENCY SECONDARY TO AUTOIMMUNITY. PRIMARY IMMUNODEFICIENCIES LEADING TO PHAGOCYTE DEFICIENCY INCLUDE CHRONIC GRANULOMATOUS DISEASE AND LEUKOCYTE ADHESION DEFICIENCY SYNDROME. SELECTIVE IMMUNOGLOBULIN A DEFICIENCY IS ALSO A PRIMARY IMMUNODEFICIENCY. SECONDARY CAUSES OF IMMUNODEFICIENCY INCLUDE STEROIDS, NUTRIENT DEFICIENCY, OBESITY, ACQUIRED IMMUNE DEFICIENCY SYNDROME (AIDS), OR OTHER VIRAL INFECTIONS. THIS ACTIVITY REVIEWS THE DIFFERENT IMMUNOLOGICAL DISORDERS, THEIR EVALUATION, AND MANAGEMENT, AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN CARING FOR AFFECTED INDIVIDUALS.