final-immune deficiencies Flashcards
opportunistic organisms take hold in the host when?
immune system is depleted
Primary Immune Deficiencies (PIDs) are clinically manifested during?
first years of life (>6 months)
PIDs are classified based on Host-defense mechanisms involving?
Ab deficiencies, T cell deficient, SCID, deficiencies of phagocytes, complement deficiencies.
Maternal and protect the infant for only?
6 months post natally
HIV, DiGeorge syndrome, CD3gamma chain mutations, and X-linked hyper IgM syndrome is due to?
T cell immunodeficiency
differential count in blood cells for B cell detects?
CD19 and CD20
lab test serum IgG, IgM, and IgA screens for humoral immunodeficiency and looks for
decrease in Ig’s
X-linked agammaglobulinemia and autosomal recessive type, IgA deficiency, Wiskott-Aldrich are types of?
B cell deficiencies
ADA- adenosine deaminase deficiency, is a
B cell progenitor deficiency- but can affect all cell types
RAG 1, RAG 2, and Artemis mutation affect?
Pro- B cells
BTK, BLNK, Ig-alpha chains affect?
Pre-B cells
CD 19 mutations are selective for
BCR on mature B cells
Failure to express Ig Receptor in developing B cells can induce?
apoptosis
X-linked agammaglobulinemia is caused by mutations in ____which disrupts rearrangement of the Ig Heavy chains. Resulting in early B cell arrest in the Pre-B cell stage. Clinically, low or absent numbers of B cell antibodies IgG, IgA, IgM)
BTK gene (Bruton Tyrosine Kinase)
X-linked agammaglobulinemia exhibits heterogeneity and has poorly developed or absent
spleen, tonsils, adenoids, Peyer’s patches, and peripheral lymph nodes
Autosomal recessive agammaglobulinemia is a mutation in?
BLNK-signal transduction pathways.
some IgG production happens in the mature B cells. Isotype switching with active ___ induces plasma cell differentiation in infections.
CD19
defects in B cell formation
inherited
antibodies against B cells for immune targeting is
acquired.
in IgA deficiency, what is not detectable?
soluble, circulating IgA’s
in hyper IgM syndromes, you have high levels of IgM but low?
IgG and IgA
X-linked hyper IgM syndromes (HIGM) are due to mutations in the? which sex is affected more?
CD40 ligand expressed on Cd4+ T helper cells which is a trigger for isotype switching and somatic hypermutation.
males- 75% for CD40ligand
Females and males- 25% autosomal CD40
transient hypogammaglobulinemia of infancy, instrinsic Ig production is delayed for up to 36 months and results in low IgG and IgA concentraionts but normal IgM.
sinopulmonary infections
low IgM, normal IgG, elevated IgA and IgE are characteristics of?
Wiskott-Aldrich syndrome