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32 Cards in this Set
- Front
- Back
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How are immunodeficiencies classified in general? How is congenital immunodificiencies catergorized?
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1. Congenital vs. Acquired (Primary vs. Secondary)
2. Abnormalities in completment, phagocytes, humoral immunity, cellular immunity |
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C1q inhibitor deficiency
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Hereditary angioedema
Continuous complement activation and consumption (overactivated classical pathway) Susceptible to SLE and immune-complex diseases |
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Decay accelerating factor (DAF/ CD55)
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Paroxysmal nocturnal haemoglobulinuria
Lysis of RBC |
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C1, C2, or C4 deficiency
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Immune complex disease (unable to remove Ag-Ab complexes)
C2 deficiency associated with SLE |
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C3 deficiency
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The most serious complement deficiency
Repeated infections with pyogenic bacteria |
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MAC (C5~C8) deficiency
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Meningococcal infections (meningitis) by Neisseria (which is the most significant human pathogen)
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Nutropenia
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Too few neutrophils
Defect in stem cell differentiation |
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Leukocyte adhesion deficiency-I and -II (LAD-I/ -II)
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Lack of adhesion to endothelium for migration into tissues
Defect in CD 18 of integrins, aka LFA(TypeI) or selectin (Type II) |
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Chediak-Higashi sndrome
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Phagosome doesn't fuse with lysosome
Defective phagocytosis and/ or natural killer (NK) cell function |
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Chronic granulomatus disease
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Defect in genes for NADPH oxidase of lysosomes
Defective intracellular bacteria killing --> Granuloma formation |
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Severe Combined Immunodeficiency (SCID)-two types
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Defects in lymphocyte maturation
1) X-linked SCID (50%): defect in a signaling subunit of a receptor for cytokines, particular IL-7 2) Autosomal: Mutation in adenoisine deaminase (breakdown of purines) or cytokine receptors Defect in T and B-cell maturation |
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Bruton's disease
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No or few antibodies
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Congenital agammaglobulinanemia aka Bruton's disease
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Mostly X-linked: due to defective gene encoding a tyrosine kinase (btk) involved in activation of pre-V cell to immature B cell --> little or few antibodies
T cells normal Little boys with big infections |
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Hyper-IgM syndrome
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Incrased IgM but little or no IgG in the circulation
Defective gene coding for either CD40 on B cells or CD40L on activated T cells |
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Common variable immunodeficiency (CVID)
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IgG or IgA deficiency
1) B cells do not undergo terminal differentiation; deficiency most common IgA (1/700 people) 2)B cells normal but T cell signaling defective |
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Transient hypogammaglobemia
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B cells normal
No CD4+ T cell help early in life |
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DiGeorge syndrome
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Defect in thymus embryogenesis or lack of thymus
Selective defects in T cell maturation |
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Which is the most common immunodeficiency? Acquired or congenital?
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Acquired
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What factors to contribute to acquired immunodeficiency (severe and mild)?
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Malnutrition, Tumors, Therapy
Aging, Trauma (glucocorticoids), Immunosuppression by microbes (HIV), Leukemia, Myeloma |
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What are the treatment options of immunodeficiency?
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1. Antibiotics - by far, most common
2. Antibodies - periodic injection of pooled immunoglobulins from normal human serum 3. Bone marrow transplant 4. Gene therapy- ideal |
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How would you evaluate antibody-mediated immunity?
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1. Serum immunoelectrophoresis
2. Quantitation of immunoglobulins in serum and secretions 3. Detection of specific abs, e.g. Schick test for anti-diphtheria antitoxin IgG 4. Quantitation of circulating B-cells: flow cytometry 5. Induction of B-lymphoc differentiation in vitro 6. Lymph node biopsy |
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How would you evaluate cell-mediated immunity?
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1. DTH skin to common antigens, e.g. candida, streptokinase, streptodornase
2. Total lymphocyte count 3. Use mAbs to CD3, CD4 and CD8 4. Lymphocyte proliferation to lectins and alloantigens 5. In vitro analysis of T-cell func.: IFN-gamma, IL-2 production |
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How would you evaluate complement system?
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1. Total hemolytic complement assay-CH50 a functional assay
2. Immunoassay of individual complement components 3. Neutrophil chemotaxis assay using C in patient's serum as a chemoattractant |
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How would you evaluate phagocytic function?
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1. Total granulocyte or monocyte count
2. Chemotaxis assay- Boyden chamber 3. Assay for phagocytosis using opsonized particles and for bacterial killing 4. Superoxide generation 5. Response to activation by IFN-gamma, GM-CSF 6. Individual enzyme assay: IL-1, IL-12 7. Antigen processing and presentation |
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Frequent myobacterial infection is an indication of what type of defect in phagocytes?
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Defects in IFN-gamma (upregulate MHCII in APCs or other cells) or IL-12 (differentiation to Th1)receptors
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Immune thrombocytopenic purpura (ITP)
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Platelets are opsonized by IgG, and the complexes are removed by Fc receptors and or complement receptor-mediated phagocytosis.
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Autoimmune hemolytic anemia (AIHA)
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Red blood cells are opsonized by IgG, and the complexes are removed by Fc receptors and or complement receptor-mediated phagocytosis.
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Myasthenia gravis (MG)
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Antibodies to the acetylcholine receptor destroy the cell or cross-linking receptor of the muscle cells. Defects in a small subset of T or B cells.
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Systemic Lupus Erythematosus (SLE)
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Autoantibodies form precepitating immune complexes which results in tissue damage, such as lysis of erythrocytes.
Kidney damage due to the deposition of immune complexes in glomeruli |
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Grave's disease
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Antibodies cause endocrine dysfuncion.
Abs to the TSH receptors on cells stimulate thyroid gland and make excessive T4/T3. |
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Hashimoto's thyroditis
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Autoantibodies and T cells cause endocrine dysfunction.
Autoabs and T cells inhibit thryoid fn. |
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Steven-Johnson syndrom
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Autoimmune disease characterized by rash, blister and epidermal detachment.
Life-threatening; drug-induced More common in Han chinese |
