Clinical Correlations test 1 Flashcards
No MHC= no adaptive immunity
SCID - die before the age of 5 years.
Bare lymphocyte sydrome
No TAP expression (what takes antigen into ER to be added onto MHC)= no MHCI
chronic respiratory infections
HSV ptoyrin
inhibits TAP function
Adeno virus protein
Inhibits MHC1 expression
Psoriasis treatment
anti-TNF antibodies
Remicade= InfliXIMAB= mouse+ human
Humira= adalimUMAB= human
9 year old boy. Recurrent URT infections and pneumonia. Onset 10months- 9years (current). Tests show low IgG, IgM and no IgA. Normal WBC.
x-linked agammaglobinemia. Doesn’t make any mature B-cells due to defect in BRUTON TYROSINE KINASE. Reduced pre-B cells in bone marrow, no mature B-cells.
Treatment for x-linked agammaglobinemia?
IV immunoglobins. Treat infections
Significance of onset of sx at 10 months?
mother Ab (IgG) runs out around 9-10months. Breast feeding confers IgA, but that will only stay in the GI tracts (won't be found in serum).
No tonsils in x-linked agamma globinemia?
They’re there. But, majority of tonsils are B-cells (germinal centers) so they’re tiny now. Not visible with naked eye. Still has T-cells there.
Treatment for systemic lupus erythematosus
Immune complex formation causes damage seen. Use anti-BAFF drug= Belimumab, Benlysta,
5-year old boy. recurrent sinus infections since 1 year. low IgG, no IgA, normal WBC. Found IgM+/IgD+ cells. DTP booster without Ab. O blood type with type A/B Ab
Hyper IgM syndrome= CD40L mutation
IgM+ or IgD+ cells chosen with RNA alternative splicing. To switch to IgA or IgG, isotype switching is needed. This patient has an isotype switching problem. Ab to blood because those are IgM and he has those.
Hyper IgM syndrome will show what when looking at lymph nodes?
No germinal center!
Why do kids <5 years have lots of URTs?
Polysaccharide is a common antigen on bacteria. It is TI-2 antigen than can induce response without T-cell. Kids t have this response yet= increased susceptibility
Conjugate polysaccharide vacciens
Haemophilus Influenza (HIB)
Streptococcus pneumoniae
Neisseria mengitidis
*protection to children <5 years because they lack polysaccharide IT2 response**
Strep pneumoniae
G+, coccobacillus
Cause: UR disease, ear infection, pneumonia, meningitis and systemic disease.
>90 serotypes. 7 and 13 valent conjugate vaccines exist.
How many days until Ab are detected following initial vaccine?
5-7 days.
Say a person is missing part of their CD3 complex, what happens?
CD3 complex is needed for TCR to leave the ER and go to the membrane. Low CD(epsilon)/gamma etc will mean less TCR= immunocompromised
SCID
No TCR = no B-cells or t-cells. More prone to infection by opportunistic pathogens. Can be due to RAG error.
DiGeorge’s Syndrome
No thymus. T-cell development doesn’t happen==> increased chances of infection (similar sx to SCID)
Bone marrow transplant in leukemia patients. What happens if there is a complete MHCI/II mismatch?
Wrong MHC in thymic epithelial cells and peripheral APC=
T-cells that are positively selected using host thymic cells can’t recognize antigens on DONOR MHCs
Thymic involution
Old people have small thymuses (thats not the only thing that shrinks….)
DR3/DR4 (HLA issues)=
systemic lupus
DR4 (HLA issues)=
Rheumatoid arthritis
B7 and DR2 (HLA issues)=
MS
