Clinical Correlations test 1

Question 1

No MHC= no adaptive immunity

Answer 1

SCID - die before the age of 5 years.

Question 2

Bare lymphocyte sydrome

Answer 2

No TAP expression (what takes antigen into ER to be added onto MHC)= no MHCI

chronic respiratory infections

Question 3

HSV ptoyrin

Answer 3

inhibits TAP function

Question 4

Adeno virus protein

Answer 4

Inhibits MHC1 expression

Question 5

Psoriasis treatment

Answer 5

anti-TNF antibodies
Remicade= InfliXIMAB= mouse+ human
Humira= adalimUMAB= human

Question 6

9 year old boy. Recurrent URT infections and pneumonia. Onset 10months- 9years (current). Tests show low IgG, IgM and no IgA. Normal WBC.

Answer 6

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.

Question 7

Treatment for x-linked agammaglobinemia?

Answer 7

IV immunoglobins. Treat infections

Question 8

Significance of onset of sx at 10 months?

Answer 8

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).

Question 9

No tonsils in x-linked agamma globinemia?

Answer 9

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.

Question 10

Treatment for systemic lupus erythematosus

Answer 10

Immune complex formation causes damage seen. Use anti-BAFF drug= Belimumab, Benlysta,

Question 11

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

Answer 11

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.

Question 12

Hyper IgM syndrome will show what when looking at lymph nodes?

Answer 12

No germinal center!

Question 13

Why do kids <5 years have lots of URTs?

Answer 13

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

Question 14

Conjugate polysaccharide vacciens

Answer 14

Haemophilus Influenza (HIB)
Streptococcus pneumoniae
Neisseria mengitidis
*protection to children <5 years because they lack polysaccharide IT2 response**

Question 15

Strep pneumoniae

Answer 15

G+, coccobacillus
Cause: UR disease, ear infection, pneumonia, meningitis and systemic disease.
>90 serotypes. 7 and 13 valent conjugate vaccines exist.

Question 16

How many days until Ab are detected following initial vaccine?

Answer 16

5-7 days.

Question 17

Say a person is missing part of their CD3 complex, what happens?

Answer 17

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

Question 18

SCID

Answer 18

No TCR = no B-cells or t-cells. More prone to infection by opportunistic pathogens. Can be due to RAG error.

Question 19

DiGeorge’s Syndrome

Answer 19

No thymus. T-cell development doesn’t happen==> increased chances of infection (similar sx to SCID)

Question 20

Bone marrow transplant in leukemia patients. What happens if there is a complete MHCI/II mismatch?

Answer 20

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

Question 21

Thymic involution

Answer 21

Old people have small thymuses (thats not the only thing that shrinks….)

Question 22

DR3/DR4 (HLA issues)=

Answer 22

systemic lupus

Question 23

DR4 (HLA issues)=

Answer 23

Rheumatoid arthritis

Question 24

B7 and DR2 (HLA issues)=

Answer 24

MS

Question 25

B8, DR3/DR4 (HLA issues)=

Answer 25

Type 1 Diabetes

Question 26

B27 (HLA issues)=

Answer 26

Ankylosing spondylitis

Question 27

Drug that inhibits T-cell proliferation? How?

Answer 27

Cyclosporin A and FK 506

block NFAT activation= blocked IL2 production

Question 28

Commonly seen in TB (just learned about it in micro)

Answer 28

granulomas. Body is trying to wall off infection. Mainly macrophages but can contain other cells. Sometimes due to poor TH1 response.

Question 29

Tuberculoid Leprosy

Answer 29

Th1 cells make IFN gamma–> intracellular pathogens destroyed=== survival, milder damage

Question 30

Lepromatous leprosy

Answer 30

TH2 cells can’t activate macrophage===unchecked proliferation of intracellular bacteria= tissue destruction, DEATH

Question 31

IPEX/XLAAD

Answer 31

From FOXP3 issues. AKA Treg, Regulatory t-cells.

Question 32

Stress? Sex hormones?

Answer 32

Negative regulators. Immunosuppression with glucocorticoids and catecholamines