Immunodeficiency Flashcards
primary immunodeficiency
inherited genetic abnormality and can arise from mutation; nature of defect determines phenotype
acquired immunodeficiency
due to environmental exposure, viruses, chemical agents, radiation
recurrent bacterial infections indicates _
B cells
recurrent viral infections indicates _
T cells
X-linked agammaglobulinemia (Bruton’s)
Btk deficiency prevents B cell development leading to recurrent bacterial infections
treatment for Bruton’s
infusion of fresh-frozen plasma or gamma globulin
acquired hypogammaglobulinemia
body slowly stops making antibodies but it is not due to decreased levels of immunoglobulin-secreting cells
CD40 ligand deficiency (X-linked hyper IgM)
defect in T cells prevents B cells from undergoing a class switch and IgM is produced at elevated levels; no IgA, IgE, or IgG
treatment for CD40 ligand deficiency
bone marrow transplant
DiGeorge syndrome
lack of thymus;
MHC class II deficiency
defect in antigen presenting cells leads to loss of CD4+ cells
treatment for MHC class II deficiency
bone marrow transplant
MHC class I deficiency
loss of Tap1 prevents transport of peptides to ER for class I binding which leads to defect in all nucleated cells plus absence of CD8+ cells
SCID
ADA or PNP deficiency causes accumulation of nucleotide metabolites which damages T cells
X-linked SCID (SCID with B cells)
gamma chain of IL-2 receptor is lost which leads to loss of function of many cytokines
