Immunodeficiency Disorders Flashcards
4 Major Components of the Immune System
B Cells
T Cells
Phagocytes
Complement
State in which the immune system’s ability to fight infectious disease is compromised
Immunodeficiency
Recurrent infections with pyogenic bacteria indicate a
B-cell Deficiency
Recurrent infections with fungi, viruses, or protozoa indicate a
T-cell Deficiency
Genetic susceptibility to infections since childhood
Primary Immunodeficiency
Acquired susceptibility to infection as a result of external processes or diseases
Secondary Immunodeficiency
Very low levels of all immunoglobulins; Virtual absence of B cells due to tyrosine kinase mutation; Cell-mediated immunity is normal
X-linked Agammaglobulinemia or Bruton’s Agammaglobulinemia
Failure of isotype switching; Recurrent bacterial sinus and lung infections
Selective IgA Deficiency
Defect in B cell maturation to plasma cells; Diagnosis of exclusion; Recurrent pyogenic bacterial infections; Most common form of severe antibody deficiency affecting both children and adults
Common Variable Immunodeficiency
Profound deficit of T cells; Failure of development of thymus and parathyroids; Humoral immunity is normal; Tetany due to hypocalcemia
Di George Syndrome
Di George Syndrome
Cardiac defect (TOF) Abnormal facies Thymic aplasia Cleft palate Hypocalcemia 22q11.2 chromosomal deletion
Specific T-cell deficiency for Candida albicans; Other T-cell and B-cell functions are normal
Chronic Mucocutaneous Candidiasis
X-linked; Defect in IL-2 receptors in T-cells; Autosomal: ADA deficiency; Recurrent bacterial, viral, fungal and protozoal infection in early infancy
Severe Combined Immunodeficiency (SCID)
X-linked; Inability to mount IgM response; Mutation in WASP gene for actin filament assembly; Recurrent pyogenic infections, eczema, and bleeding due to thrombocytopenia
Wiskott-Aldrich Syndrome
Wiskott-Aldrich Syndrome
Thrombocytopenia
Infections
Eczema
WASP mutation
