Immunodeficiency Syndromes

Question 1

Defects in T cell function predispose host to IC (viral) infections. True or false?

Answer 1

True

Question 2

What do defects in B cell function and Ig production predispose animals to?

Answer 2

EC bacterial disease

Question 3

Why are combined immunodeficiences severe?

Answer 3

Because affected animals lack resistance to ALL infectious agents

Question 4

What is the difference between congenital and acquired immunodeficiencies?

Answer 4

Congenital (primary) affects the innate and adaptive immune system and is genetic.

Acquired (secondary) is cause by microbial infections.

Question 5

How are genetic disorders inherited?

Answer 5

Autosomal recessive inheritance happens when parents carry the gene defect.

X-Linked inheritance occurs as a result of mutations in the X chromosome.

Question 6

The is no male to male transmission in X-linked inheritance. True or false?

Answer 6

True

Question 7

Leukocyte adhesion deficiency type I- innate or adaptive disorder?

Answer 7

innate, autosomal

Question 8

What molecule is affected in leukocyte adhesion deficiency type I and why is this critical?

Answer 8

CD18 becomes non-functional.

CD18 is the integrin critical for leukocyte adhesion and migration into the tissues, when it is non-functional leukocytes cannot migrate to site of infection.

Question 9

What are Bovine LAD and Canine LAD?

Answer 9

leukocyte adhesion deficiency type I affecting cattle and dogs

Question 10

What molecule is affected in leukocyte adhesion deficiency type II and why is this critical?

Answer 10

Defect is in L-selectin.

Selectin molecules interact with leukocytes to slow them down to allow them to migrate into infected tissues.

Question 11

Chediak-Higashi syndrome- innate or adaptive disease?

Answer 11

innate, autosomal

Question 12

What molecule is affected in Chediak-Higashi syndrome and why is this critical?

Answer 12

LYST is affected

Prevents lysosome from fusing to phagosome containing trapped pathogen.

Question 13

What cells are affected by Chediak-Higashi syndrome?

Answer 13

neutrophils, macrophages, DCs, NK cells, cytotoxic T cells, etc

Question 14

What species are most affected by Chediak-Higashi syndrome?

Answer 14

cats and cattle

Question 15

What are the consequences of Chediak-Higashi syndrome?

Answer 15

susceptibility to diseases and early death

Question 16

What is the affected molecule in Chronic granulomatous disease and why is it critical? Innate or adaptive?

Answer 16

Innate

Phagocyte oxidase complex Phox-19 affected. Prevents the phagoytes from making reactive oxygen species making them useless because that is their killing mechism.

Question 17

What is affected in TLR signaling defects?

Answer 17

CD40

PAMPs cannot be sensed, resulting in host being unable to signal the rest of the body that there is an active infection

Question 18

Complement deficiences make up what percentage of all primary immunodeficiencies?

Answer 18

1-10%

Question 19

What is affected in complement defiencies?

Answer 19

C5-C9, meaning MAC is not successfully assembled

Question 20

What cells are effected by SCID?

Answer 20

T-cells, B-cells, and NK cells

Question 21

What does SCID result in?

Answer 21

very low circulating lymphocytes

failure to synthesize Igs

hypoplasia of lymphoid tissues due to lack of Ig

Question 22

What is defective in SCID?

Answer 22

autosomal: defect in Protein Kinase (DNA-PKcs)

X-linked (XSCID): defect in gamma chain of IL-2Ry

Question 23

What is the result of XSCID?

Answer 23

Decreased T-cells and NK cells

Normal number of B-cells with decreased function

Question 24

What is the result of autosomal SCID?

Answer 24

Decreased number of T and B cells

End result failure of VDJ recombination, no BCR diversity

Question 25

What is CVID?

Answer 25

Common Variable ID

Question 26

What is mutated in CVID? What does this affect?

Answer 26

ICOS and TACI Affects the ability to produce gammaglobulins

Question 27

How does CVID affect B-cells?

Answer 27

Lack of blood cells in blood, bone marrow, or spleen

Question 28

What animals are usually affected by CVID?

Answer 28

>3 yo horses miniature dachshunds

Question 29

SCID- innate or adaptive?

Answer 29

adaptive

Question 30

CVID- innate or adaptive?

Answer 30

adaptive

Question 31

Hyper-IgM syndrome- innate or adaptive

Answer 31

adaptive

Question 32

Agammaglobulinemic- innate or adaptive?

Answer 32

adaptive

Question 33

Thymic hypoplasia- innate or adaptive?

Answer 33

adaptive

Question 34

Selective immunoglobulin deficiency- innate or adaptive?

Answer 34

adaptive

Question 35

What is mutated in Hyper-IgM syndrome and what does it affect?

Answer 35

CD40, CD40L, or NEMO Affects T-dependent B-cell activation, APC activation, and cell-mediated immunity

Question 36

What is agammaglobulinemia?

Answer 36

B cell deficiency

Question 37

What is mutated in x-linked agammaglobulinemia?

Answer 37

B-cell Tyrosine Kinase (BTK) gene, prevents the ability of B-cells to develp, differentiate, and activated.

Question 38

What is mutated in autosomal agammaglobulinemia?

Answer 38

Mutations in IgM heavy chain resulting in inability to make functional BCR

Question 39

What is used to treat CVID in humans?

Answer 39

Gamunex, purified gamma globulin IgG from donated blood

Question 40

What are some examples of causes of acquired IDs?

Answer 40

HIV Protein-calorie malnutrition Irradiation and chemotherapy Cancer metastases Immunosuppression for transplants Removal of spleen

Question 41

What cells does infectious bursal disease virus affect?

Answer 41

B-cells

Question 42

What cells do FIV and SIV affect?

Answer 42

Viruses affecting T-cells, retroviruses

Question 43

What cells do bovine diarrhea virus affect?

Answer 43

macrophages

Question 44

What cells do porcine reproductive and respiratory syndrome virus affect?

Answer 44

Macrogphages

Question 45

Colostrum deprivation, vitamin A and D deficiency, and factors such as exercise and age can cause acquired IDs. True or false?

Answer 45

True