Congenital and Acquired Immunodeficiencies Flashcards
(37 cards)
what is another name for Wiskott Aldrich Syndrome?
eczema-thrombocytopenia immunodeficiency
what antibodies are high in WAS?
IgA/IgE
what cells are abnormal in WAS?
B cells
What is the Treatment for WAS?
bone marrow transplant
What is deficient in X-linked SCID?
y (gamma) chain in the cytokines: IL-2/4/7/9/15
what is affected in the other 2 autosomal SCID’s?
1) ADA
2) PNP
what cell lineage is mostly affected in SCID?
T cells don’t mature
what 2 symptoms are found in SCID?
no T helper cells
chronic baby diarrhea
no thymus develops
In autosomal SCID-ADA what lymphocytes are deficient?
T cells
B cells
NK cells
In autosomal SCID-PNP, what lymphocytes are absent?
no T cells
in X-Linked SCID, what lymphocytes are absent?
T cells and therefore no B cells
what is absent in DiGeorge?
The thymus
what 3 signs are seen in DiGeorge?
1) abnormal jaw
2) abnormal aorta
3) abnormal parathyroid glands
If there is a defect in the transport of MHC 1 molecules to the surface of the cells, I have this disease…
TAP deficiency
what cells can’t load MHC 1 into their surface in TAP deficiency?
all nucleated cells
what 2 things are mainly seen in TAP deficiency?
1) necrotizing granulomas
2) nose and face have skin lesions
What lymphocyte is absent in XLA (brutton’s)
B cells
what is the treatment for XLA?
and IV of Immunoglobulin’s IgA/IgE/IgM
what happens if a polio vaccine is given to a kid with XLA?
he might suffer paralysis
What physical thing is absent in XLA (brutton’s)?
tonsils
what auto-antibodies are seen in IgA deficiency?
there may be autoantibodies against IgA
how are IgA deficiency patients treated?
you treat specific infections
what is deficient in X-linked Hyper IgM?
the CD-40L
In X-linked hyper IgM, what cells can’t communicate? what happens do to this lack of communication?
t helper cell with B cell; t cell cant tell b cell to class switch
