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Immuno > Immunodeficiencies > Flashcards

Immunodeficiencies Flashcards

1
Q

What are immunodeficiencies?

A

a lack or failure of the immune system

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2
Q

What are primary immunodeficiencies? Secondary?

A 
Primary = congenital
Secondary = acquired
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3
Q

What is the most common cause of immunodeficicencies worldwide?

A

Malnutrition

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4
Q

What type of infections occur more often in a defect humoral immunity?

A

Extracellular bacteria

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5
Q

What type of infections occur more often in a defect cell mediated immunity?

A

Viruses and intracellular bacteria

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6
Q

What type of infection occur more often when there is a defect in both humoral and cell mediated immunity?

A

All types

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7
Q

Immunodeficient individuals are more susceptible to developing certain kinds of cancers. What is the etiology of these CA?

A

Stem from viral infections

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8
Q

What is X-linked agammaglobinemia?

A

Absence of B cells due to a defect in B cell Tyrosine kinase

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9
Q

What broad class of people usually gets X-linked agammaglobinemia?

A

Males

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10
Q

What type of infections are pts with X-linked agammaglobinemia susceptible to?

A

Pyogenic bacteria of extracellular origin

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11
Q

What is diagnostic for x-linked agammaglobinemia?

A

Absence of circulating B lymphocytes

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12
Q

Why can you not screen infants for x linked agammaglobinemia at 6 months?

A

Still have mother’s antibodies

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13
Q

How do you treat x-linked agammaglobinemia?

A

Monthyl IVIG

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14
Q

Why aren’t pts with x-linked agammaglobinemia more susceptible to viruses or fungal infections?

A

T cells intact.

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15
Q

What is common variable immunodeficiency?

A

an inability of mature B lymphocytes to differentiate into plasma cells

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16
Q

Does common variable immunodeficiency affect males and females equally?

A

Yes

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17
Q

How do you diagnose common variable immunodeficiency?

A

normal levels of circulating mature B lymphocytes which can proliferate in response to antigen, but do not differentiate into plasma cells.

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18
Q

Is common variable immunodeficiency acquired or congenital?

A

Either

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19
Q

What is the etiology of common variable immunodeficiency?

A

Unknown, but may be d/t intrinsic B cell defects

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20
Q

What is the treatment of common variable immunodeficiency?

A

monthly gamma globulin shots and antibiotic therapy PRN

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21
Q

What are the comorbidities of common variable immunodeficiency?

A

Predisposed to autoimmune diseases

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22
Q

What is selective IgA deficiency?

A

Inability to produce IgA antibodies

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23
Q

Is selective IgA deficiency inherited or acquired?

A

Either

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24
Q

What are the symptoms of selective IgA deficiency?

A

usually asymptomatic, but blood transfusions can be problematic

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25
Q

Why are blood transfusions problematic for pts with selective IgA deficiency?

A

Pts will produce anti-IgA antibodies since they were first exposed to it via breast milk. Causes hyperacute reaction

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26
Q

Why is selective IgA usually asymptomatic?

A

IgM makes up for it

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27
Q

Why can IgM make up for the lost IgA in selective IgA deficiency?

A

Has a J chain, and allows it to be transported across mucosal lining

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28
Q

What is the treatment for selective IgA deficiency?

A

Antibiotics to control infections at mucosal sites

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29
Q

Why can you not give IVIG to pts with IgA deficiency?

A

They will form hyperacute reaction to the antibodies

30
Q

How do you diagnose IgA defciency?

A

Severely decreased levels of IgA.

31
Q

True or false: pts with IgA deficiency do not express B cells with IgA antibody on their cell surface

A

False–they do, but not known why these do not differentiate into plasma cells

32
Q

What are the cytokines that are suspected to play a role in selective IgA deficiency?

A

IL-5 and TGF-beta.

33
Q

What are the comorbidities of selective IgA defciencies?

A

allergies, GI tract, and autoimmune diseases

34
Q

What is hyper IgM syndrome?

A

“All IgM, all the time” with NO other antibody types produced

35
Q

What are the two types of hyper IgM syndrome?

A

x-linked

Hyper IgM type II syndrome

36
Q

What is the cause of X-linked hyper IgM syndrome?

A

Lack of CD-40 ligand on T cells

37
Q

What is the cause of hyper IgM type II syndrome?

A

inherited mutation in the gene activation-induced cytidine deaminase which, for unknown reasons, prevents isotype switching.

38
Q

What is the defective chromosome in Di-george syndrome?

A

22

39
Q

What is the immune problem in Di-george syndrome?

A

Lack of thymus resulting in lack of T cell production

40
Q

What are the facial features of Di-George syndrome?

A

Low set ears and fish mouth

41
Q

What pharengeal arches does the thymus arise from?

A

3-4

42
Q

Di-George syndome predisposes pts to what type of infections?

A

viral and fungal infections

think cell mediated

43
Q

Are immunoglobin levels normal in pts with Di-george syndrome? Why?

A

Typically yes, because TI antigens still a significant fraction of antibodies

44
Q

What is the “treatment” for Di-George syndrome?

A

Thymus transplant, but if pt survives long enough, will generate T cells of unknown origin

45
Q

What are SCID?

A

Defects in both B and T lymphocytes

46
Q

True or false: the spleen, lymph nodes, tonsils, and appendix are not populated by B and T lymphocytes in pts with SCID

A

True

47
Q

Why can you not give immunizations with live, attenuated vaccines to pts with SCID?

A

Will cause infection and death

48
Q

What are the four molecular bases of SCID?

A
  1. Lack of adenosine deaminase
  2. Lack of purine nucleotide phosphorylase (PNP)
  3. RAG1/2 gene defects
  4. Mutations for cytokine receptors IL-2,4,7,9,15.
49
Q

A family h/o early death from infections would be alarming for what disease?

A

SCID

50
Q

What is the treatment for SCIDs?

A

Bone marrow transplant (or retroviral therapy)

51
Q

What is chronic Granulomatous disease?

A

Recurrent bacterial infections d/t a defect in NADPH oxidase in PMNs

52
Q

What are the treatments for chronic granulomatous disease?

A

Bone marrow transplant

IFN-gamma therapy

53
Q

What is myeloperoxidase deficiency?

A

decreased or absent myeloperoxidase enzyme needed to produce HOCl, used by PMNs as a ROS

54
Q

What type of infections are pts with myeloperoxidase deficiency susceptible to?

A

There is no increase in susceptibility to infection is seen in these individuals due to compensatory immune mechanisms. However, there can be a decreased ability to ward off certain fungal infections (particularly Candida yeast).

55
Q

What is Chediak-Higashi syndrome?

A

Congenital disorder leading to recurrent bacterial infections.

56
Q

What is the molecular basis of Chediak-Higashi syndrome?

A

lysosomes of PMNs fuse, reducing their ability to kill microbes

57
Q

Is T cell and/or NK cell function impaired in pts with Chediak-Higashi syndrome?

A

Yes

58
Q

What are the signs of Chediak-Higashi syndrome? (4)

A
  1. Silver hair
  2. Platelet abnormalities
  3. vision impairment
  4. PMNs have larger granules
59
Q

What is the treatment for Chediak-Higashi syndrome?

A

Bone marrow transplant

60
Q

What is Leukocyte adhesion deficiency-1 (LAD-1)?

A

defect in the beta chain of LFA-1 and Mac-1 adhesion molecules renders neutrophils incapable of adhering to endothelial cells for extravasation and suppresses chemotaxis.

61
Q

What are the symptoms of Leukocyte adhesion deficiency-1 (LAD-1)?

A

Recurrent bacterial and fungal infection

62
Q

What is the treatment for Leukocyte adhesion deficiency-1 (LAD-1)? (3)

A
  1. Antibiotics PRN
  2. Granulocyte infusions
  3. Bone marrow transplant
63
Q

What is diagnostic for Leukocyte adhesion deficiency-1 (LAD-1)?

A

Extreme leukocytosis /bc PMNs cannot extravasate

64
Q

NOT tested but important:

What is severe congenita neutropenia and cyclic neutropenia? Molecular cause?

A

Either none or cyclical amounts of PMNs. Caused by a defect PMN elastase enzyme

65
Q

What is Wiskott-Aldrich syndrome?

A

Poor lymphocyte response to antigens

66
Q

What is the pattern of inheritance for Wiskott-Aldrich syndrome?

A

X-linked

67
Q

What is the triad of symptoms for Wiskott-Aldrich syndrome?

A
  1. Eczema
  2. Thrombocytopenia
  3. Immunodef
68
Q

What are the comorbidities of Wiskott-Aldrich syndrome?

A

Autoimmune diseases

69
Q

What is the molecular basis of Wiskott-Aldrich syndrome?

A

defective cytosolic protein (Wiskott-Aldrich Syndrome protein – WASP) that helps regulate the actin cytoskeleton of bone marrow-derived cells

70
Q

What is the treatment for Wiskott-Aldrich syndrome?

A

IVIG and abx. Bone marrow transplant

71
Q

What is the molecular basis for Ataxia-telangiectasia?

A

ATM defect, thus cannot regulate cell cycle

Immuno (16 decks)

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