the immune system is compromised, it’s weaker or · 2020. 12. 20. · the immune system is...
TRANSCRIPT
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The immune system is compromised, it’s weaker or DEFECTED
We’ll talk first about the primary then we’ll have a brief look at the secondary type.
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The common thing between the 130 disorders is that there’s a mutation in the development of lymphocytes caused the impairment.
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The doctor only followed the route of the map: hemayopoeitic cells give common lymphoid progenitor and on and on.
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AKA 22q11 , because it’s caused due to a deletion in chromosome 22 (next slide)
Problems in T cells
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The 3rd & 4th pharyngeal arches are involved in the development of the thymus.
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The main manifestation observed in Digeorge patients is the absence of a thymus ! Therefore they’ll have a deficiency in the development of T cells
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But you have to be smart, T cells deficiency doesn’t always mean DiGeorge syndrome, you have to consider the other manifestations.
The 3rd & 4th pharyngeal arches are also involved in the development in the heart & some B.Vs
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T cells are releasing cytokines but the receptor for these cytokines is defected
So neither T nor B cells will maturate
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Toxic to lymphocytes-> kill them (B&T cells)
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We can’t give them attenuated live vaccines
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These stem cells are aquired from totally healthy pateins with no defects in any of things we’ve mentioned before, hoping that these cells will generate normal lymphocytes
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You don’t have to memorise anything from the table above, just keep in mind that multiple genes are involved and can cause this disease
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A mutation in a gene that codes a tyrosine kinase that plays a key rule in the maturation of B cells -> no B cells no immunoglobulins
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All you need to know is that there’s a problem in the somatic recombination of the light chains
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It’s more common in males because it’s a X-linked disease, one allele will cause it
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No problem in antiviral / antifungal responses
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A mutation to an autosomal chromosome rather a sex chromosome (not x linked)
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X-linked diseases are more common in males
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