Immunology Flashcards
State in which the immune system’s ability to fight infectious disease is compromised
Immunodeficiency
4 Major Components of the Immune System
B CellsT CellsPhagocytesComplement
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 faciesThymic aplasiaCleft palateHypocalcemia22q11.2 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
ThrombocytopeniaInfectionsEczemaWASP mutation
Autosomal recessive disease; Mutations in DNA repair enzymes; IgA deficiency; Ataxia, Telangiectasia, recurrent infections by 2 years of age
Ataxia-Telangiectasia
Lack NADPH oxidase activity; Failure of oxidative burst; Normal B and T cell activity; Recurrent infections with: catalase (+) bacteria and fungi (A. fumigatus); Widespread granulomas of unknown etiology
Chronic Granulomatous Disease
Autosomal recessive disease; Failure of Phagolysosomal Fusion; Faulty microtubules impair neutrophil chemotaxis; Recurrent pyogenic infections caused by Staphylococci and Streptococci
Chediak-Higashi Syndrome
Autosomal recessive diseases; Mutation in integrins; Defective adhesion (LFA-1) proteins on the surface of phagocytes; Severe pyogenic infections in infancy; Delayed separation of umbilical cord
Leukocyte Adhesion Deficiency (LAD)
Most common complement defect; Usually asymptomatic but may develop septicemia or SLE
C2 Deficiency
Recurrent pyogenic infections due to Staphylococcus aureus
C3 Deficiency
Specific deficiency in C5-C9; Inability to form membrane-attack complexes; Severe bacteremia with Neisseria meningitidis or Neisseria gonorhoeae
Terminal Complement Deficiency
B Cell Deficiency
Bruton’s XLAIgA Deficiency
T-cell Deficiency
Di George Syndrome
B-Cell and T-Cell Deficiency
Severe Combined Immunodeficiency Wiskott-Aldrich SyndromeAtaxia Telangiectasia
Phagocyte Deficiency
Chronic Granulomatous DiseaseLeukocyte Adhesion DeficiencyChediak-Higashi Syndrome
Complement Deficiency
C5-C9 Deficiency
Decreased supply of amino acids; Decreased synthesis of IgG and Complement
Malnutrition
HIV infects and kills CD4+ helper T-cells; Loss of cell-mediated immunity
AIDS