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Immunology > Lecture 30: Immunodeficiencies > Flashcards

Lecture 30: Immunodeficiencies Flashcards

1
Q
A
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2
Q

Immunodeficiencies result from the ____

A

Absence or failure of normal function of immune system

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3
Q

What is the primary consequence of immunodeficiencies

A

Increased susceptibility to infection

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4
Q

What type of infections by organisms do not commonly cause disease in immunocompetent animals

A

Recurrent or chronic infections

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5
Q

What is the common infectious consequence of B cell deficiencies

A

Bacterial infections

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6
Q

What is the common consequence of T cell deficiencies

A

Viral and intracellular microbial infections

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7
Q

What is the common consequence of innate immune deficiencies

A

Bacterial and viral infections

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8
Q

What type of immunodeficiency is inheritable and develop in young animals

A

Primary (genetic)

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9
Q

What type of immunodeficiency is acquired and tend to occur in adult animals

A

Secondary

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10
Q

Primary immunodeficiencies can result in defects in __maturation, ___ activation or components of ___immunity

A

Lymphocyte maturation, lymphocyte activation and innate immunity

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11
Q

Innate immune deficiency defects typically occur in what processes

A

Phagocytosis and intracellular killing or complement

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12
Q

What complement involves C1, C4 and C2 to form C3 convertase

A

Classical

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13
Q

What pathway involve sMASP, C4, and C2 to form c3 convertase

A

Mannose binding lectin

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14
Q

What pathway involves C3, CFB, and CFD to form c3 convertase

A

Alternative

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15
Q

What is the most severe complement innate deficiency

A

C3 deficiency

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16
Q

What complement deficiency results in severe recurrent bacterial infections usually in skin and respiratory systems

A

C3 deficiency

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17
Q

What complement deficiency results in increased C3a and C5a convertase activity

A

Factor H deficiency

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18
Q

Factor H deficiency results in increased ___

A

Inflammation

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19
Q

What gene is affected in Chediak Higashi Syndrome

A

LYST (CHS1)

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20
Q

What doe LYST regulate

A

Intracellular protein trafficking of lysosomes and secretory granules

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21
Q

What does a mutation of LYST result in

A

Abnormally large granules:

Lysosomes of leukocytes and platelets
Secretory granules of various cells
Melanosomes of melanocytes

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22
Q

What symptoms characterize chediak higashi syndrome

A

Albinism, photophobia, peripheral neuropathy, bleeding disorders, increased infections

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23
Q

What genetic mutation causes Pelger Huet Anolay

A

Lamin B receptor (LBR)

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24
Q

What is the result of a mutation in LBR

A

Failure of granulocyte nuclei to segment into lobes (hypo segmentation)

Granulocytes appear immature, left shift on CBC, may be mistaken for early stage lymphoma

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25
Q

PHA is generally lethal in what type of inheritance

A

Homozygous

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26
Q

A mutation in what gene causes trapped neutrophil syndrome

A

VPS13B

27
Q

What does a mutation in VPS13B cause

A

Failure of bone marrow to release neutrophils resulting in severe neutropenia

28
Q

What characterizes trapped neutrophil syndrome

A

Long head and legs, failure to thrive, weakness, joint pain and swelling, recurrent bacterial infections

29
Q

Leukocyte adhesion deficiencies is caused by what

A

Deficiency in Cd18-integrity B2 chain

30
Q

What is the result of leukocyte adhesion deficiency

A

Impaired WBC extravasation resulting in high WBC counts

31
Q

What mutation causes cyclic neutropenia

A

AP3B1

32
Q

What does a mutation in AP3B1 result in

A

Shorter PMN life span, cyclic PMN counts, recurrent bacterial infections, joint pain and bleeding disorders

33
Q

What is the genetic mutation in foal immunodeficiency syndrome

A

Sodium/myo inositol co-transporter SLC5A3

34
Q

What is the result of a mutation in SLC5A3

A

Low levels of RBC’s and B cells, become progressively anemic and more susceptible to recurrent infections

35
Q

What is the mutation in XLA

A

Btk

36
Q

What does mutation in Btk cause

A

Inability of Pre-B cells to synthesis and express complete BCR, resulting in B cell apoptosis

37
Q

What disease is characterized by a lack of B cells and Ig’s in serum and results in recurrent bacterial infections

A

XLA

38
Q

IgA deficiency results in what

A

Inability to differentiate into IgA secreting plasma cells which results in recurrent infections on mucosal surfaces

39
Q

What Ig deficiency is correlated with higher rates of allergies

A

IgA

40
Q

Immunoglobulin deficiencies can cause what problem in foals

A

Failure of passive transfer

41
Q

What gene is mutated in thymus aplasia

A

FOXN1

42
Q

What is FOXN1 involved in

A

Regulation of T cell maturation

43
Q

Thymus aplasia is characterized by what

A

Reduced T cells production resulting in increased susceptibility to opportunistic infections

44
Q

What mutation is associated with congenital hypotrichosis (no hair growth)

A

FOXN1

45
Q

Severe combined immuno-deficiencies results in deficit in what cells

A

B and T lymphocytes

46
Q

Patients with severe combined immuno-deficiencies are highly susceptible to

A

Life-threatening viral, bacterial, and fungal infections

47
Q

What is DNA-Pk involved in

A

VDJ recombination process in both B and T cells

48
Q

What does a mutation in DNA-pk result in

A

Failure of B and T cell maturation resulting in severe lymphopenia

49
Q

What SCID causes patients to lack all serum Ig’s, hypoplasia of lymphatic tissues and severe infections beginning at 6-8 weeks of age

A

Mutation in DNA-PK

50
Q

What is the common y subunit a component of

A

Cytokine receptors

51
Q

A mutation in y subunit results in

A

Nonfunctional cytokine receptors which are important for lymphocyte maturation and function

52
Q

How is y subunit mutation characterized

A

Poorly developed thymus, lack of T cells, lack of IgG/IgA (IgM okay), failure to thrive and severe infections around 6-8 weeks of age

53
Q

Malnutrition shows increased susceptibility to infection due to impairment of what cell

A

T cell function

54
Q

Effects of malnutrition have thought to been related to levels of what hormone

A

Leptin

55
Q

Malnutrition results in decrease ___ cells and release of immunosuppressive cytokines such as __

A

T cells, IL-10

56
Q

Over nutrition activates what macrophages and results in what

A

M1- release of pro-inflammatory cytokines

57
Q

What drug has numerous immunosuppressive effects

A

Glucocorticoids

58
Q

What drug inhibits NFAT and therefore T cell production

A

Cyclosporine

59
Q

FIV primarily affects and destroys what cells and results in what

A

CD4T resulting in decline of cell mediated immunity and increased susceptibility to infection

60
Q

BLV infects and transforms what cells

A

B cells

61
Q

Chronic stress leads to elevation of endogenous ___ which results in what

A

Glucocorticoids, immunosuppression

62
Q

Stress induced immune suppression plays a major role in susceptibility to ____ disease

A

Infectious

63
Q

Describe the decline in immune function with age

A

Immunosenescence

Immunology (22 decks)

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