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30 Cards in this Set
- Front
- Back
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what are the 2 types of immunodeficiency |
primary (congenital) resulting from genetic defects - inherited. Deficiency causes disease
secondary (acquired) - result of other diseases or conditions » HIV infection » malnutrition » immunosuppression |
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what is primary immunodeficiency also known as |
congenital |
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what is secondary immunodeficiency also known as |
acquired |
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are immunodeficiencies normally dominant or recessive |
recessive |
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what are primary (congenital) immunodeficiencies present as in young |
primary (congenital) immunodeficiencies are commonly present as recurrent infection in young |
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what are primary immunodeficiencies also important for |
allergy preventing autoimmunity controlling proliferation of abnormal lymphocyte proliferation preventing cancer |
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list the different deficiencies that can occur as a result of primary immunodeficiency |
Humoral/ B cell defects Cell-mediated/ T cell defects Faulty phagocytes Complement deficiency |
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what different defects can occur as a result of Humoral/ B cell defects in primary immunodeficiencies |
-Bruton's (X-linked) Aggamaglobulinaemia -hyper IgM syndrome -IgA deficiency |
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what is the underlying problem in Bruton's (X-linked) Aggamaglobulinaemia |
block in maturation beyond pre-B cells because of mutation in B cell tyrosine kinase |
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what 3 things is there a reduction in as a result of Bruton's (X-linked) Aggamglobulinaemia |
-decrease in all serum Ig isotypes -reduced B cell numbers -few follicles in lymph nodes |
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what are the main problems that occur as a result of Hyper IgM syndrome |
-high IgM serum due to defective B cell heavy chain isotype switching -mutation in CD40 ligand where Th cell binds to B cells/macrophages -susceptible to infection |
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what is the most common immunodeficiency syndrome |
IgA deficiency |
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explain how cell-mediated/T cell defects occur |
B cells rely on signals from T cells to work properly B cells function normally in vitro but not in vivo due to lack of signals from T cells Humoral immunity may be preserved |
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what is X-linked SCID |
severe combined immunodeficiency |
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what does SCID result in in terms of B cells and T cells |
decreased T cells B cells normal |
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where are the mutations in SCID |
mutations in cytokine receptors "IL-2Rγ" chain
codes for interleukin 2 receptor |
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give an example of a primary congenital disease in which there are faulty phagocytes |
chronic granulomatous disease |
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what causes chronic granulomatous disease |
defects in NADPH enzymes that generate superoxide radicals (reactive oxygen species-ROS) which are involved with killing bacteria
the reason this is a disease which involves faulty phagocytes is because the immune system then tries to compensate by recruitment of more phagocytes, therefore leading to the accumulation of phagocytes around the foci of infection |
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what is the rarest of the immunodeficiencies |
complement deficiency |
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in complement deficiency what are the symptoms |
the symptoms will depend on where the pathway defect occurs
-may involve regulatory proteins |
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what are the different types of secondary (acquired) immunodeficiency |
-removal of spleen -cancer metastasis to bone marrow -HIV to AIDs -chemotherapy for cancer -protein-calorie malnutrition |
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why does the spleen need to be removed in some types of secondary (acquired) immunodeficiency |
decreased phagocytosis of microbes |
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why does cancer metastasis of bone marrow in some types of secondary (acquired) immunodeficiency occur |
reduced site of WBC production |
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why does protein-calorie malnutrition occur in secondary (acquired) immunodeficiency |
metabolic derangements inhibit lymphocyte maturation and function |
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why is chemotherapy for cancer required after secondary (acquired) immunodeficiency |
decreased bone marrow precursors for all leukocytes |
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HIV is a secondary (acquired) immunodeficiency, how does it infect the cells of the immune system |
HIV is a retrovirus that infects the cells of the immune system, mainly CD4+ T lymphocytes, and causes progressive destruction of these cells |
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where does the primary infection of cells take place in AIDs |
primary infection is of the cells in blood mucosa |
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where does the infection get established in AIDs |
lymphoid tissue (nodes) |
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upon infection in the lymphoid tissue (nodes), what happens next |
immune response |
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what happens after the immune response in AIDs and how does this lead to the destruction of lymphoid tissue along with the associated depletion of CD4+ cells |
clinical latency - establishment of chronic infection the virus gets trapped in lymphoid tissue by follicular DCs which leads to low level virus production destruction of lymphoid tissue along with associated depletion of CD4+ cells this leads to opportunistic diseases attacking the now weak immune system and then cell death |
