MODULE IV Flashcards
what are the examples of inappropriate and pathologic responses?
immunodeficiency (primary, secondary)
hypersensitivity
gammopathy
autoimmune disease or immunopathology
what are the types of immunodeficiencies?
primary or genetic secondary or acquired cells and systems involved -phagocyte (nonspecific dysfunction) -complement (non/specific dysfunction) -humoral (specific dysfunction) -cell mediated (specific dysfunction)
what are the warning signs of immune deficiency?
2 or more ep of pneumonia 4 of more episodes of otitis recurrent stomatitis recurrent abcess 1 episode of severe systemic infection recurrent intestinal infection severe asthma adverse affect to BCG clinical phenotype suggestive family history
effect of B cell deficiencies?
bacterial infections
effect of T cell deficiencies?
viral and other intracellular microbial infections
innate immune deficiencies?
variable; pyogenic bacterial infections
chronic granulomatous disease
name this immune system deficiency
absent or deficient expression of B2 integrins causing defective leukocyte adhesion dependent functions
leukocyte adhesion deficiency 1
name this immune system deficiency
absent or deficient expression of leukocyte ligands for endothelial E and P selectins, causing failure of leukocyte migration into tissues
leukocyte adhesion deficiency 2
name this immune system deficiency
defect in complement cascade activation
complment C3 deficiency
name this immune system deficiency
deficient activation of classical pathway of complement leading to failure to clear immune complexes and development of lupus like disease
complement of C2, C4 deficiency
name this immune system deficiency
defective lysosome function
Chediak-Higashi syndrome
what type of defect is this?
recurrent non superficial pyogenic infections and recurrent ENT and airway infections
phagocytic cell defects
name this phagocytic cell defect
this is a killing defect where there is a lack of NADPH oxidase (therefore decrease ROS)
chronic granulomatous disease (CGD)
name this phagocytic cell defect
this is a chemotaxis defect with high IgE, staph infections, lung disease, eczema, rare
hyper IgE syndrome (Job syndrome)
name this phagocytic cell defect
chemotaxis defect with defects in neutrophil chemotaxis, adhesion, and activation issues
leukocyte adhesion defect (LAD)
name this phagocytic cell defect
chemotaxis defect in which there is a failure to thrive
schwachman-diamond syndrome
name this phagocytic cell defect
chemotaxis defect with ROS
chediak higashi syndrome
what are the immunologic and nonimmunologic characteristics of Hyper-IgE syndrome?
immunologic characteristics
- newborn rash
- boils
- eczema
- elevated IgE
- eosinophilia
nonimmunologic characteristics
- characteristic face
- retained primary teeth
- hyperextensibility
defect in leukocyte adhesion and migration responsible for neutrophilia or neutropenia
Leukocyte adhesion deficiency (LAD)
mutation in SBDS gene diarrhea, failure to thrive, malabsorption til 4 mo eczema, recurrent infections neutrophil could < 1000, pancytopenia risk of AML
Shwachman Diamond Syndrome
autosomal recessive disorder characterized by partial occulocutaneous albinism, grayish white hair, giant granules in many cells, neutropenia and recurrent infections
chromosomal mutation 1q43 affecting Lyst gene
chediak higashi syndrome
a type of cytotoxic lymphocyte that is not class restricted?
these play a major role in rejection of tumors and cells infected by viruses
the cells kill by releasing small cytoplasmic granules of proteins causing apoptosis
natural killer cells
affect of classical NK cell deficiency?
Qty in peripheral circulation
affect of functional NK cell deficiency?
function deficiency