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Kaplan Qbank USMLE



Author9 Posts
  #1

HI guys,
I cant understand Bare Lymphocyte syndrome. can anaybody explain it to me?

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WE MIGHT HAVE COME FROM DIFFERENT PLACES, BUT WE ARE HEADING IN THE SAME DIRECTION

  #2

Its MHC class 2 deficiency...now MHC 2 helps in forming T helper cells so these cells are deficient in this syndrome....so immune probelms...earliest one to present with this is SCID...and other opportunistic infections can occur like HIV
Important thing to remember is that there is no GVHD as MHC 1 antigens are expressed normally

  #3

bare lymphocyte syndrome is MHC class II deficiency, therefore, these patients are deficient in CD4+ cells because of failure of positive selection in the thymus.

they will have moderate to severe hypogammaglobulinemia.

MHC class I is expressed normally so they do have CD8+ cells, but their function is diminished by abscence of TH1 cell cytokines.

early on bare lymphocyte synd. presents as a mild form of SCID, however it can be cleary distinguished from true SCID in that these patients will have T cells that can respond to nonspecific T-cell mitogens.




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Every disaster hides an opportunity.

  #4

thanks for both of you. you are great

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WE MIGHT HAVE COME FROM DIFFERENT PLACES, BUT WE ARE HEADING IN THE SAME DIRECTION

  #5

just to mention that, to distinguish SCID and bare lymphocyte syn.,in bare lympho there is proliferation of B cells after mitogen stimulation where as in SCID there is no response.

  #6

Thank you


  #7

what is a mitogen ? can anybody please tell me the mechanism of action of a mitogen

  #8

and please tell also how there is no GVHD .its very confusing for me.
thanx in advance

  #9

IF MHCI are present(they are on all the recepients cells) then CD8+ of the donor will recognize them in the recepient and will attack them.ON THE OTHER HAND if MHCII are present they are only on APCs , which in number they r not compared to the number of all cells in the body. from that i think that MHCI in the recepient influence more the GVHD than MHCII.
from that my question is how in bare lymphocyte syndrome there's no GVHD ,though MHCI are expressed normally..?????????????????







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