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IMMUNOLOGY > Lecture 30: Immunodeficiencies > Flashcards

Lecture 30: Immunodeficiencies Flashcards

1
Q

2 types of immunodeficiencies

A

Primary - genetic
Secondary - acquired

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2
Q

characteristics of primary immunodeficiency

A
  • develop in VERY young animals (<6mo) when maternal protection wanes
  • typically involve genetic mutations
  • rare conditions
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3
Q

Characteristics of secondary immunodeficiency

A
  • tend to occur in adult animals
  • can be transient or permanent deficiencies
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4
Q

which type of immunodeficiency is more common

A

secondary

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5
Q

what can primary immunodeficiency result from

A

defects in lymphocyte maturation, lymphocyte activation/function, defects in innate immunity

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6
Q

patients with innate immune deficiencies are especially susceptible to infections from…

A

encapsulated bacteria

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7
Q

deficits of innate immunity typically occur during what processes of cell defense

A

phagocytosis, intracellular killing, or proteins of the complement system

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8
Q

severe recurrent bacterial infections are typically observed in _____ complement deficiency

A

C3

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9
Q

most severe of the complement deficiencies

A

C3
- skin and respiratory most common

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10
Q

Factor H (CFH) Deficiency causes

A

increased inflammation (Glomerulonephritis)
- common in yorkshire pigs
- increases C3&C5 convertase activtiy

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11
Q

Chediak-Higashi Syndrome

A
  • **defect in gene LYST (CHS1) **which regulates intracellular protein trafficking of lysosomes and secretory granules
  • -mutation interferes w/ synthesis and function of granules
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12
Q

Chediak-Higashi Syndrome is characterized by

A
  • albinism
  • photophobia
  • peripheral neuropathy
  • bleeding disorder
  • increased infections
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13
Q

Pelger-Huet Anomaly (PHA)

A
  • mutation in LBR=lamin B receptor
  • failure of granulocyte to segment into lobes (nuclear hyposegmentation)
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14
Q

Trapped Neutrophil Syndrome

A
  • mutation in VPS13B
  • Border Collies
  • mutation results in failure of bone marrow to release neutrophils, results in severe neutropenia
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15
Q

Characteristics of Trapped Neutrophil Syndrome

A
  • abnormally long head and legs
  • failure to thrive
  • weakness
  • joint pain
  • swelling
    * recurrent bacterial infections
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16
Q

Leukocyte Adhesion Deficiencies

A
  • Deficiency of CD18= integrin B2 chains, results in impaired extravasation of WBCs
  • results in high WBC counts and reccurent bacterial infection
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17
Q

Cyclic neutropenia (gray collie syndrome)

A

mutation in AP3B1 encoding neutrophil elastase, results in decreased enzyme activity

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18
Q

patients with B cell deficiencies are more susceptible to…

A
  • some bacteria
  • parasites
  • viruses in which immunity is primarily antibody mediaated
19
Q

F

Foal Immunodeficiency syndrome is a ____ cell deficiency

A

B

20
Q

Foal Immunodeficiency syndrome

A
  • mutation of the sodium/myo-inositol cotransporter gene, SLC5A3
  • mainly affects Fell and Dales ponies
  • SLC5A3 plays a role in regulation cellular responses to osmotic stress
21
Q

Characteristics of Foal Immunodeficiency syndrome

A

abnormally low RBCs and B cell levels
as levels drop, foals become progressively more anemic and susceptible to recurrent infections

22
Q

X-linked Agammaglobulinemia (XLA)

A
  • mutation in gene encoding Bruton’s tyrosine kinase (Btk)
  • results in inability of pre-B cells to synthesize and express complete RBC, results in apoptosis of pre-B cells
23
Q

characteristics of XLA

A
  • lack of B cells, lack of Igs in serum, no follicles or germinal centers in lymphoid tissues
  • recurrent bacterial infections (commonly resp and GI)
24
Q

A common Ig deficiecny is selective ____ deficiency

A

IgA

25
Q

What is selective IgA deficiency

A

inability of B cells to differentiate into IgA-secreting plasma cells
leads to recurrent infections on mucosal surfaces, higher allergy rates

Shar pei and German shepherds

26
Q

T-cell Deficiencies cause patients to be more susceptible to..

A
  • viruses
  • intracellular microorganisms
  • many opportunistic pathogens
27
Q

Patients w/ Tcell deficiencies are usually also deficient in _______ and _______ immune responses

A

cell-mediated immunity and humoral immune responses

28
Q

Thymic Aplasia

A

mutation in gene FOXN1 invovled in the regulation of Tcell maturation

29
Q

Thymic Aplasia is characterized by…

A

reduced Tcell production
increased susceptibility to opportunistic infections
congenital hypotrichosis (no hair growth)

30
Q

is deficiency in B or T cells more severe

A

T cells
bc regulators of immune system and help assist activation of B cells

31
Q

what is SCID

A

Severe combined immunodeficiencies
severe deficit in the quantity and or functions of BOTH B and T lymphocytes
signs of disease occurs after maternal production wanes

32
Q

SCID patients are highly susceptible to…

A
  • life threatening viral, bacterial and fungal infections early in life
33
Q

Mutation in DNA-Dependent Protein Kinase (DNA-PK)

A

autosomal recessive
DNA-PK is involved in VDJ recombination process in both B and T cells
enzyme involved in ligating the DNA breaks, resulting in a lack of both B and T cell maturation

34
Q

T/F: a mutation in DNA-PK that prevents VDJ recombination is preventing B and T cells from synthesizing their specific antigen receptors

A

T

35
Q

DNA-PK characteristics

A
  • severe lymphopenia
  • lacks of all serum Igs, hypoplasia of lymphatic tissues
  • severe infections beginning around 6-8 weeks
36
Q

Mutation in the common y subunit of cytokine receptors

A

common y subunit is a component of several cytokine receptors
lack of this renders cytokine receptors non-functional
cytokines are important in lymphocyte maturation & function

37
Q

Characteristics in common y subunit mutation

A

poorly developed thymus
lack of T cells
lack of serum IgG/IgA
severe infections beginning around 6-8 weeks

38
Q

most common causes of secondary immunodeficiences

A
  • malnutrition & overnutrition
  • medical immunosuppression
  • infections
  • chronic disease
  • stress
  • immunosenescence
39
Q

severe malnutrition increases susceptibility to infection due to …..

A

impairement of T cell function

40
Q

impairment of T cell function caused by malnutrition is related to levels of…?

A

leptin = adipokine related to body fat mass

less body fat = less leptin

41
Q

patients with decreased leptin levels have an increased susceptibility to infection due to …

A
  • decreased T cell numbers and function
  • increase in immunosuppressive cytokines (IL-10)
42
Q

obese tissue is rich in what type of macrophages

A

Classically activated M1

43
Q

large quantities of leptin stimulates the production of _____ cytokines

A

pro inflammatory

IMMUNOLOGY (26 decks)

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