Immunodeficiency Syndromes Flashcards

1
Q

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

A

True

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2
Q

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

A

EC bacterial disease

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3
Q

Why are combined immunodeficiences severe?

A

Because affected animals lack resistance to ALL infectious agents

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4
Q

What is the difference between congenital and acquired immunodeficiencies?

A

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

Acquired (secondary) is cause by microbial infections.

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5
Q

How are genetic disorders inherited?

A

Autosomal recessive inheritance happens when parents carry the gene defect.

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

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6
Q

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

A

True

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7
Q

Leukocyte adhesion deficiency type I- innate or adaptive disorder?

A

innate, autosomal

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8
Q

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

A

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.

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9
Q

What are Bovine LAD and Canine LAD?

A

leukocyte adhesion deficiency type I affecting cattle and dogs

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10
Q

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

A

Defect is in L-selectin.

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

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11
Q

Chediak-Higashi syndrome- innate or adaptive disease?

A

innate, autosomal

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12
Q

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

A

LYST is affected

Prevents lysosome from fusing to phagosome containing trapped pathogen.

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13
Q

What cells are affected by Chediak-Higashi syndrome?

A

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

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14
Q

What species are most affected by Chediak-Higashi syndrome?

A

cats and cattle

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15
Q

What are the consequences of Chediak-Higashi syndrome?

A

susceptibility to diseases and early death

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16
Q

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

A

Innate

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

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17
Q

What is affected in TLR signaling defects?

A

CD40

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

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18
Q

Complement deficiences make up what percentage of all primary immunodeficiencies?

A

1-10%

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19
Q

What is affected in complement defiencies?

A

C5-C9, meaning MAC is not successfully assembled

20
Q

What cells are effected by SCID?

A

T-cells, B-cells, and NK cells

21
Q

What does SCID result in?

A

very low circulating lymphocytes

failure to synthesize Igs

hypoplasia of lymphoid tissues due to lack of Ig

22
Q

What is defective in SCID?

A

autosomal: defect in Protein Kinase (DNA-PKcs)

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

23
Q

What is the result of XSCID?

A

Decreased T-cells and NK cells

Normal number of B-cells with decreased function

24
Q

What is the result of autosomal SCID?

A

Decreased number of T and B cells

End result failure of VDJ recombination, no BCR diversity

25
Q

What is CVID?

A

Common Variable ID

26
Q

What is mutated in CVID? What does this affect?

A

ICOS and TACI

Affects the ability to produce gammaglobulins

27
Q

How does CVID affect B-cells?

A

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

28
Q

What animals are usually affected by CVID?

A

> 3 yo horses

miniature dachshunds

29
Q

SCID- innate or adaptive?

A

adaptive

30
Q

CVID- innate or adaptive?

A

adaptive

31
Q

Hyper-IgM syndrome- innate or adaptive

A

adaptive

32
Q

Agammaglobulinemic- innate or adaptive?

A

adaptive

33
Q

Thymic hypoplasia- innate or adaptive?

A

adaptive

34
Q

Selective immunoglobulin deficiency- innate or adaptive?

A

adaptive

35
Q

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

A

CD40, CD40L, or NEMO

Affects T-dependent B-cell activation, APC activation, and cell-mediated immunity

36
Q

What is agammaglobulinemia?

A

B cell deficiency

37
Q

What is mutated in x-linked agammaglobulinemia?

A

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

38
Q

What is mutated in autosomal agammaglobulinemia?

A

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

39
Q

What is used to treat CVID in humans?

A

Gamunex, purified gamma globulin

IgG from donated blood

40
Q

What are some examples of causes of acquired IDs?

A

HIV

Protein-calorie malnutrition

Irradiation and chemotherapy

Cancer metastases

Immunosuppression for transplants

Removal of spleen

41
Q

What cells does infectious bursal disease virus affect?

A

B-cells

42
Q

What cells do FIV and SIV affect?

A

Viruses affecting T-cells, retroviruses

43
Q

What cells do bovine diarrhea virus affect?

A

macrophages

44
Q

What cells do porcine reproductive and respiratory syndrome virus affect?

A

Macrogphages

45
Q

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

A

True