Immunopathology III Flashcards
(53 cards)
immunodeficiency disorder invovle ______ defects
adaptive or innate immune system
What are immunodefiency disorders divided into
primary and secondary types
what does immunodefieciency usually present as
freequence, severe, resistant infections
no symptoms free interval
unusual etiologic organism
severe, unexpected complications
primary immunodefiencies
generally rare, genetically determined defects of B and T lymphocytes
clinical manifestations of primary immunodeficiencyes
6mons - 2 years
repeated infection
failure to thrive
X-linked agammagloulinemia of bruton
failure of normal pre-B cells to undergo further differentiation, causes an absense of mature B cells
when does X-linked agammagloulinemia of bruton arise
after depletion of maternal Ab
x-linked agammaglouliemia may be viewed as a disorder of waht
opsonization
how do treat x-linked agammagloulinemia of bruton
parenteral immunoglobulin replacement
how is cellular immunity affected by x-linked agammagloulinema of bruton
intact, but susceptibiliiyt of enteric virus or protozoa from a lack of neutralizing IgA
common variable immunodefiency
fairly common, heterogenous group of disorders with sporadic and inferited forms
main feature of common variable immunodeficiency
hypogammaglobulinema, usuallyu all Ab classes but occasionally isolated IgG
who gets common variable immunodefiency
equal amoung men and women
happens in 2nd -3rd decades
mechanisms of common variable immunodeficiency
intrinsic B cell defect
abnormal T cell signal to B cell
what does lack of IgA in common variable immunodefiency cause
incrased entervoiral infection, chronic diarrhea
what disorders are increased in common variable immunodifern
AI disroders
lymphioids and gastric cacners
Selective IgA defiency is caused by
IgA being absent of IgA +B cells don’t mature
what is selective IgA deficiency assocaited with
family history
measles
toxoplasmosis
40% of people with IgA definecy have what
anti-IgA Ab
what do you have to keep in mind with blood transfusions and IgA defiency
can ahve an anaphylactic reaction if the transfused blood tehy are given contains IgA
hyper IgM syndrome
disorder characterized by failure of T cells to induce B cell isotype switching
activate macrophages to remove intracellular microbes
what do IgM-producing B cells normally do
activate transcription genes that encode for other Ig isotypes via signals between CD40 molecule on B cell and CD40 ligand on helper T cell
how is IgG affected by hyper IgM syndrome
decreased or absent
how is hyper IgM syndrome transmitted
x linked or autosomal recessive
