HEREDITARY SPHEROCYTOSIS

Course overview

HEREDITARY SPHEROCYTOSIS, A TYPE OF CONGENITAL HEMOLYTIC ANEMIA, IS THE MOST PREVALENT CAUSE OF HEMOLYTIC ANEMIA DUE TO AN ABNORMAL RED CELL MEMBRANE. THESE ATYPICAL ERYTHROCYTES ARE UNABLE TO MAINTAIN THEIR NORMAL BICONCAVE SHAPE DUE TO GENETIC MUTATIONS IN THE MEMBRANE/CYTOSKELETAL PROTEINS THAT PLAY A ROLE IN STRUCTURAL MORPHOLOGIC STABILITY. THE CLINICAL MANIFESTATIONS VARY ACCORDING TO THE SEVERITY OF THE DISEASE. THIS ACTIVITY OUTLINES THE EVALUATION AND MANAGEMENT OF HEREDITARY SPHEROCYTOSIS AND EXPLAINS THE ROLE OF THE INTERPROFESSIONAL TEAM IN PROVIDING QUALITY CARE.