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32 Cards in this Set

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  • Back
How are immunodeficiencies classified in general? How is congenital immunodificiencies catergorized?
1. Congenital vs. Acquired (Primary vs. Secondary)
2. Abnormalities in completment, phagocytes, humoral immunity, cellular immunity
C1q inhibitor deficiency
Hereditary angioedema
Continuous complement activation and consumption (overactivated classical pathway)
Susceptible to SLE and immune-complex diseases
Decay accelerating factor (DAF/ CD55)
Paroxysmal nocturnal haemoglobulinuria
Lysis of RBC
C1, C2, or C4 deficiency
Immune complex disease (unable to remove Ag-Ab complexes)
C2 deficiency associated with SLE
C3 deficiency
The most serious complement deficiency
Repeated infections with pyogenic bacteria
MAC (C5~C8) deficiency
Meningococcal infections (meningitis) by Neisseria (which is the most significant human pathogen)
Too few neutrophils
Defect in stem cell differentiation
Leukocyte adhesion deficiency-I and -II (LAD-I/ -II)
Lack of adhesion to endothelium for migration into tissues
Defect in CD 18 of integrins, aka LFA(TypeI) or selectin (Type II)
Chediak-Higashi sndrome
Phagosome doesn't fuse with lysosome
Defective phagocytosis and/ or natural killer (NK) cell function
Chronic granulomatus disease
Defect in genes for NADPH oxidase of lysosomes
Defective intracellular bacteria killing --> Granuloma formation
Severe Combined Immunodeficiency (SCID)-two types
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
Bruton's disease
No or few antibodies
Congenital agammaglobulinanemia aka Bruton's disease
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
Hyper-IgM syndrome
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
Common variable immunodeficiency (CVID)
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
Transient hypogammaglobemia
B cells normal
No CD4+ T cell help early in life
DiGeorge syndrome
Defect in thymus embryogenesis or lack of thymus
Selective defects in T cell maturation
Which is the most common immunodeficiency? Acquired or congenital?
What factors to contribute to acquired immunodeficiency (severe and mild)?
Malnutrition, Tumors, Therapy
Aging, Trauma (glucocorticoids), Immunosuppression by microbes (HIV), Leukemia, Myeloma
What are the treatment options of immunodeficiency?
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
How would you evaluate antibody-mediated immunity?
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
How would you evaluate cell-mediated immunity?
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
How would you evaluate complement system?
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
How would you evaluate phagocytic function?
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
Frequent myobacterial infection is an indication of what type of defect in phagocytes?
Defects in IFN-gamma (upregulate MHCII in APCs or other cells) or IL-12 (differentiation to Th1)receptors
Immune thrombocytopenic purpura (ITP)
Platelets are opsonized by IgG, and the complexes are removed by Fc receptors and or complement receptor-mediated phagocytosis.
Autoimmune hemolytic anemia (AIHA)
Red blood cells are opsonized by IgG, and the complexes are removed by Fc receptors and or complement receptor-mediated phagocytosis.
Myasthenia gravis (MG)
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.
Systemic Lupus Erythematosus (SLE)
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
Grave's disease
Antibodies cause endocrine dysfuncion.
Abs to the TSH receptors on cells stimulate thyroid gland and make excessive T4/T3.
Hashimoto's thyroditis
Autoantibodies and T cells cause endocrine dysfunction.
Autoabs and T cells inhibit thryoid fn.
Steven-Johnson syndrom
Autoimmune disease characterized by rash, blister and epidermal detachment.
Life-threatening; drug-induced
More common in Han chinese