ch 18 Flashcards
what are the categories of immunodeficiencies
primary and secondary
primary immunodeficcieny
genetic
dysregulation of one or more arms of the immune system
innate
adaptive
heterogenous
secondary immunodeficiceny
caused by something
chronic ilness- cancer, diabetes, malnutrition, infection (HIV)
trauma (splendectomy, burns)
exposure- radiation, chemo (medications)
where can we find primary immune defects
enzymes
signalling molecules
cell surface protein
cytoskeleton
receptor protein
what kin of immunodeficiency/ defect is most common
B cell defects
due to B cell intrinsic deficiency or B-T interactions
immunodeficncy symptoms may reflect
intrinsic or intreractive defects
what are the examples of humoral defects
hyper igM syndrome
igA deficiency
CVID
RESPIRATORY INFECTIONS
What is at a higher risk due to humoral defects
autoimmunity and malignancy
how to treat humoral defects
replacement immunoglobulin
humoral defects
examine B cellnumber and function bc they have an impaired or poor quality production of antibodies
what is the cuase of x linked agammaglobulinemia
mutations in the brutons tyrosine kinase BTK gene
what is the function of BTK
essential for normal B cell development - absence results in absence of mature B cell and plasma cells and germinal centres
X linked agammaglobulinemia symptoms
arise once newborns passively acquired igG levels go down
leads to reccurent bacterial infections
no mature b cells
no Ab production
atypical forms w low B cell and antibody prodcution levels
UNAFFECTED T CELLS
X linked agammaglobulinemia treatment
intravenous gamma globulins
treatment of X linked hyper igM syndrome
y globulins
