Immunopathology III Flashcards
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
how are B and T cell populations affected by hyper IgM syndrome
normal
how are B and T cell populations affected by hyper IgM syndrome
normal
what type of infections tend to affect hyper IgM syndrome
pyogenic, intracellular organisms
how do you get DiGeore syndrome
partial or complete interruption of 3rd and 4th pharygneal pouches
what organs are affected by digeorger
hypoplasia of thymus nd parathyoirds
what are features of digeorge
t celld efect
hypocalcemia
cardiac abnormaliies
cleft palate
what mutation causes digeorger
deletion on 22q11
who gets Digeorge
equally male and female
how are immunoglobulins affected by DiGeorge
normal levels
facial features of digeorge
low set ears
midline cleft
small mandible
how is heart affected in digeorge
VSD
right subclaviar a derived form pulomary artery
what causes more problems with digeore - hypoplasia or aplasia
aplasia
SCID
boy in plastic buble
defieecnt in cellular and humor immune responses
how is SCID transmitted
x linked
autosomal recessive
what is the more rare from of SCID
combined T and defect
what is the more common form of SCID
severe T cell defect
x-linked SCID
mutation of common gamma chain subunit of cytokine receptors
autosomally recessive SCID
adensonine deaminase deficeincy
failure of Class ii MHC expression
clinical features of early onset SCID
thrush, diaper rash, failure to thrive
recurrent infection
how do you treat early only SCID
bone marrow transplant
how is Wiskott Aldrich transmitted
x linked
what are the feautres of wiskott aldrich
immunodefiency, thrombocytopenia, eczema
results of wiskott aldrich syndrome
Ab low or elevated
no Ab to polysacchride Ag
T cell deficent
clinical features of wiskott aldrich
hemorrhagic diathesis
recurrent respiratio infection
increased lymphoid maligancies in survivors past age 10
secondary immunodefiencyes
more common than primary
immune imparment in previously healthy people
can be reversible
what are the mechanisms of T cell loss in HIV
apostosis in affected cells
apoptosis of infected cells
CTLs kill infected cells
effect of T cell loss in HIV
lymphopenia of CD4+T cell
imparied specific B cell response to new Ag
altered macrophage fucntion with decreased MHC II expression
AIDS defining neoplasma
kaposi’s sarcoma
B cell lymphoma
primary lymphoma of brain
invasive carcinoma of uterine cervix and anus
what is the buzz word for jiroveci fugus
coffee bean
