Immunodeficiency Flashcards
What type of block is associated with low T and B cells?
block type 1- Severe combined immunodeficiency Disease (SCID)
What type of block is associated with normal T cells but low to absent B cells?
Block type 2- X-linked Bruton’s Agammaglobinemia
What type of block is associated with high IgM and low IgG and IgA
Block type 3- X-linked HyperIgM syndrome
What type of block is associated with normal numbers of pre-B cells and B cells but the B cells are difficult to trigger to switch antibody?
Block type 4- Common Variable Immundeficiency (CVID)
What type of block is associated with absent T cells and normal B cells?
Block type 5- DiGeorge Syndrome
What is the problem in block type 3?
CD40 or CD40L is defective. B cell is driven hard but can’t receive instruction to switch past making IgM
In which block type is there a defect in the stroma?
Block type 5- DiGeorge. Stroma develops abnormally and does not support lymphoid development in the thymus
What kind of genetic pattern is SCID?
X-linked recessive.
What kind of block involves a defect in the gene for the gamma chain that forms part of the receptors for IL2 and other cytokines necessary for lymphoid development?
Block 1 (SCID-X1)
B cell deficiency is characterized by infections with “high-grade” (pus-producing) bacterial pathogens such as _______
Staph. Aureus
Transient hypogammaglobulinemia children present with what type of infection?
gram positive bacterial infection
Also this condition accounts for about 15% of chronic diarrhea
What defiency is 10-15 times more frequent in those with celiac disease?
selective IgA deficiency
When would you want to use IVIG?
when B cell function is deficient. It is usually about 99% IgG.
What might be a good treatment for SCID patients?
bone marrow transplant or even better is purified stem cell transplant.
An assay for C1inh inhibitor might be used to test what?
the functioning of complement in a patient.
