X-LINKED AGAMMAGLOBULINEMIA

Course overview

X-LINKED AGAMMAGLOBULINEMIA OR XLA IS A PRIMARY IMMUNODEFICIENCY DISORDER THAT PREVENTS AFFECTED INDIVIDUALS FROM MAKING ANTIBODIES AND REQUIRES THEM TO RELY ON LIFELONG IMMUNOGLOBULIN REPLACEMENT THERAPY FOR SURVIVAL. WITHOUT IMMUNOGLOBULINS (OR ANTIBODIES), XLA PATIENTS ARE RENDERED VULNERABLE TO INVASIVE INFECTIONS. HOSPITALIZATION FOR BACTERIAL PNEUMONIA, REQUIRING INTRAVENOUS ANTIBIOTICS FOR RESOLUTION, IS USUALLY WHAT PROMPTS THE DIAGNOSTIC WORK-UP FOR PRIMARY IMMUNODEFICIENCY. THIS ACTIVITY REVIEWS THE PATHOPHYSIOLOGY OF XLA AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN ITS MANAGEMENT.