Q: List 6 functions of cell mediated immunity in host defense. A: viral immunity, fungal immunity, Ab production against T-dependent antigens, homograft rejection, protection against intracellular bacterial infections, tumor surveillance Q: What are the three tests used to determine immunity? A: delayed skin hypersensitivity, T cell quantification - different types, Mitigic assay (LAM) Q: What is SCID? A: Severe Combined Immune Deficiency Q: List 3 SCID diseases? A: 1)X-linked SCID, 2)Autosomal recessive SCID due to ADA, PNP deficiency, 3)Autosomal recessive SCID due to other causes Q: What other things can you measure to determine immune status? A: cytokine production, presence Q: What are some clinical characteristics of SCID? A: Multiple conditions; recurrent infections, viral respiratory infections, candidasis, pneumocystis, pertussis couph, diarrhea, intracellular bacti infections, pogenic bacti infections post 6 mo. Old. Q: What happens if there is a loss of ADA, adenosine deaminase? A: there is a build up of purines, to toxic levels Q: What is the significance of not having MHC class I or II expression? A: Cannot present antigens Q: What happens in Purine Metabolic Pathway Abnormalities? A: Build up of toxic products Q: Adenosine deaminase deficiency is seen in 20% of SCID patients, what does it do? A: catalyzes adenosine and deoxyadenosine to inosine and 2'deoxyinosine Q: The build up of adenosine and deoxyadenosine is toxic to what structures? A: lymphocytes Q: There is a condition in the purine metabolism pathway that results in mostly the destruction of T cells, what is it called? A: Purine Nucleoside Phosphorylase Deficiency Q: What is X-linked SCIDS A: NO T cell production, normal or increased B cell production, reduced serum Ig Q: What is the defect in X-linked SCIDS? A: mutation in gene for gamma chain of IL-2 receptor Q: What does the IL-2 receptor have to do with T cell production? A: mediates growth via multiple cytokine production, IL2, IL4, IL7, IL15 Q: Why does an X-linked SCID patient also have poor antibody production? A: T cells help instruct the production of antibodies Q: T cell maturation defects are rare, what is the most frequent disease seen? A: Di George Syndrome Q: What is Di George Syndrome? A: Thymic hypolasia Q: What is thymic hypoplasia? A: Abnormal or No thymus development, abnormality of third and fourth pharyngeal pouch, which leads to decreased T cell maturation Q: What are some characteristics that should signal Di George Syndrome (6+)? A: seizures, hypocalcemia, heart murmur, peculiar facies, cleft palate, recurrent infections Q: What is Wiskott-Aldrich Syndrome? A: combined T and B cell deficiency Q: Is Wiskott-Aldrich Syndrome X-linked? A: yes Q: List symptoms of Wiskott-Aldrich Syndrome (6+). A: can't walk or maintain balance, eczema, thrombocytopenia, bleeding, bruising, recurring infections, ataxia, telangiectasia Q: Why do you see the bruising and bleeding in the Wiskott-Aldrich Syndrome? A: poor platelet size and function, therefore poor clotting Q: What is telangiectasia? A: dilation of small or terminal vesicles, bloody rash or spots in eyes and on skin, associated with Wiskott-Aldrich Q: What is ataxia? A: inability to coordinate muscle activity Q: Why can X-raying a Wiskott-Aldrich patient result in a possible malignant tumor growth? A: they have DNA repair failures Q: What is the Bare Lymphocyte Syndrome? A: no MHC II expression Q: What deficiency is seen in Bare Lymphocyte Syndrome? A: loss of helper T cells, CD4, decrease in T cell dependent humoral immunityty