Flashcards in Immunodeficiency Deck (15)
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?
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 _______
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.