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MICRO > Ch8: Immunodeficiency and Transplantation > Flashcards

Ch8: Immunodeficiency and Transplantation Flashcards

1
Q

What is primary immunodeficiency?

A

Due to genetic mutations and are inherited

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2
Q

Types of primary immunodeficiency?

A

Dominant
Recessive
X-linked

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3
Q

What is secondary immunodeficiency?

A

Acquired/Environmental

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4
Q

What can cause secondary immunodeficiency?

A

Irradation
Drug exposure
Infection

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5
Q

Classical complement pathway deficiency results in what?

A

Immune-complex disease

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6
Q

MBL pathway deficiency results in what?

A

Bacterial infections

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7
Q

Alternative pathway deficiency results in what?

A

Infection with pyogenic bacteria and neisseria

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8
Q

Problem with C3b deposition results in what?

A

Pyogenic bacteria and Neisseria (maybe immune-complex)

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9
Q

Problem with C5 through C9 result in what?

A

Deficiency with Neisseria species

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10
Q

Problem with DAF or CD59?

A

Auto-immune conditions

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11
Q

Problem with C1INH results in what?

A

Hereditary angioneurotic edema (HANE)

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12
Q

What is the genetic of C1INH disease?

A

Autosomal dominant

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13
Q

C1INH is a member of what family of molecules?

A

Serpin protease inhibitors

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14
Q

How does C1INH work?

A

Binds to C1r or C1s and is cleaved by it.

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15
Q

HANE results in what?

A

Swelling of face, larynx and abdomen

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16
Q

HANE is commonly seen when?

A

Trips to dentist

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17
Q

Leukocyte adhesion deficiency results in what?

A

No recruitment of phagocytes to infection site resulting in widespread infections

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18
Q

What is cause of chronic granulomatous disease?

A

Defective NADPH oxidase so that macrophages can’t kill bacteria resulting in huge granulomas forming.

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19
Q

What is G6PD deficiency?

A

Defective respiratory burst so that phagocytosed bacteria can’t be killed

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20
Q

What is myeloperoxidase deficiency?

A

Deficiency in neutrophil granules and macrophage lysosomes resulting in lack of oxygen species so that phagocytosed bacteria can’t be killed.

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21
Q

What is Chediak-Higashi syndrome?

A

Defect in forming phagolysosome so results in persistent bacteria infections

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22
Q

LAD-1 deficiency is caused by what?

A

Mutations in Beta-2 integrins

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23
Q

LAD-2 deficiency is caused by what?

A

No sialyl lewis

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24
Q

Herpes simplex I is caused by what?

A

Defective antiviral immunity in CNS

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25
Q

Recurrent bacteria pneumonia is caused by what?

A

Defective innate immune response to pyogenic bacteria.

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26
Q

X-linked agammaglobulinemia (XLA) is a defect in what?

A

Btk that does intracellular signaling in B cell receptor

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27
Q

People with XLA can develop B cells how far?

A

Pre-B cell

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28
Q

Defects in ADA or PNP result in what?

A

No Pro or Pre B or T cells

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29
Q

Defects in RAG1 and RAG2 result in what?

A

No Pre B or T cells

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30
Q

Defect in Zap70 results in what?

A

No cytotoxic T cells

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31
Q

Defect in MHC class II results in what?

A

No CD4 T cells

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32
Q

IL-12 receptor deficiency results in what??

A

Recurrent intracellular bacteria like myobacterium

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33
Q

Which is worse, absence of a cytokine or a receptor?

A

Receptor because cytokines can be compensated by other cytokines

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34
Q

No IL-12 would mean what?

A

Can’t activate NK Cells

Can’t activate CTL hence Th1 cells

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35
Q

No IFN-gamma receptor would result in what?

A

Inability to clear intracellular bacteria because macrophages wouldn’t respond to IFN-gamma

36
Q

What cell signaling is interrupted with no IFN-gammareceptor?

A

Jak/STAT

37
Q

Defect in IL-2 receptor gamma signaling chain affects what?

A

Affect signaling for a ton of cytokines

38
Q

X-linked SCID is what?

A

No gamma chain on IL-2 receptor

39
Q

Autosomal recessive SCID is what?

A

Jak3, ada, pnp

40
Q

X-linked hyper IgM syndrome patients lack what?

A

CD40

41
Q

What happens in x-linked hyper IgM?

A

No CD40 so B cells are not activated and continue to release IgM at high amounts

42
Q

STAT3 mutation would result in what?

A

No Th17 cells

43
Q

No IL-12R or IFNgamma-R’s would result in what?

A

No Th1’s

44
Q

AID mutations result in what?

A

Hyper-IGM syndrome (autosomal)

45
Q

B cell deficiencies have what abnormalities?

What takes advantage?

A

No germinal centers
Reduced serum Ig levels

Pyogenic bacterial infections

46
Q

T cell deficiencies have what abnormalities? 3

What takes advantage?

A

Reduced T cell zones
Reduced DTH reactions
Defective T cell proliferation

Viruses

47
Q

Innate immune deficiencies have what consequences? (2)

A
  1. pyogenic bacteria

2. Viruses

48
Q

How long does it take for IgG to be made well enough by child?

A

1 year

49
Q

HIV has what effect on immune?

A

No CD4 T helper

50
Q

Irradiation and chemo have what effect on immune?

A

No progenitor lymphocytes

51
Q

Immunosuppression for graft rejection and inflammatory diseases cause what?

A

Less lymphocytes

52
Q

Bone marrow cancers have what effect on immune?

A

Less leukocyte development

53
Q

Protein-calore malnutrition result in what?

A

Metabolic derangements that affect lymphocytes

54
Q

Removal of spleen results in what?

A

Less phagocytosis of microbes

55
Q

Two types of rejections?

A

Host vs graft

Graft vs host

56
Q

Hyperacute rejection is the result of what?

A

Pre-formed antibodies

57
Q

Acute rejection is the result of what?

A

Direct allorecognition by pre-formed T cells

58
Q

Chronic rejection is the result of what?

A

Indirect allorecognition where a whole immune response is developed.

59
Q

What is xenograft?

A

Donor and recipient different species

60
Q

What is allograft?

A

Donor and recipient same species

61
Q

What is syngraft?

A

Donor and recipient are twins

62
Q

What is autograph?

A

Donor is recipient

63
Q

Which are generally rejected of the types of grafts?

A

Xeno and ALlo

64
Q

Rejection of organ is mediated by what? 2

A

Antibody

T cells

65
Q

What is the best donor blood?

A

O-negative

66
Q

What happens in hyperacute rejection of fetus?

A

Mother has antibodies against father’s HLA/MHC in fetus

67
Q

What is hyperacute transplant rejection?

A

Pre-formed antibodies bind to the transplant are complement cascade is activated which results in cell lysis through MAC formation

68
Q

What is a microcytotoxicity test?

A

Ab against an HLA is performed.
Complement is added to punch holes
Trypan blue is added to see if antibody actually bound and let complement punch holes.

69
Q

What is mixed lymphocyte reaction?

A

Take peripheral blood from both donor and recipient.
Irradiate one set to stop growing and then mix with the other cells.
If there is a reaction and the non-irradiated cells react to the others, then there is a mismatch.

70
Q

What is direct allorecognition due to?

A

Trying to match MHC before between donor and recipient

71
Q

What happens in direct allorecognition?

A

DC from donor tissue express MHC that is recognized by host cells but the peptides are not, so there is a response to kill foreign cells shortly after transplantation.

72
Q

How long til indirect allorecognition occurs?

A

Years

73
Q

What is indirect allorecognition?

A

Breakdown of donor MHC-expressing cells leads to recognition by B cells and T cells and the host establishes effective immune response against the graft.

74
Q

What specifically happens in both direct and indirect allorecognition?

A

Direct: Recipient T cells recognize donor MHC with the donor antigen.
Indirect: Recipient T cells do not recognize donor MHC, and must have recipient DC present antigens

75
Q

Indirect allorecognition can induce what?

A

Antibodies

76
Q

How to stop anti T cell antibodies? (2)?

A
  1. Anti-lymphocyte serum

2. Anti- T cell serum

77
Q

Three main categories of immunosuppressive drugs?

A
  1. Corticosteroids
  2. Cytotoxic drugs
  3. Microbial products
78
Q

Corticosteroids have what main response?

A

Change gene expression and shut down inflammation

79
Q

Cytotoxic drug treatment results in what?

A

Death of proliferating cells

80
Q

Microbial products have what effect in immune?

A

INhibit T cell activation?

81
Q

Three examples of microbial products?

A
  1. Cyclosporin A
  2. Tacrolimus
  3. Rapamycin
82
Q

Microbial products inhibit what?

A

T-cell receptor signaling

83
Q

When does graft versus host disease occur?

A

Following allogenic or xenogeneic bone marrow transplant

84
Q

GVHD leads to what?

A

Organ damage and death

85
Q

What are immune privileged sites?

A

Sites that tolerate graft well without an immune response such as eyes, brain, testis, and bone.

86
Q

What makes a site immune privileged?

A

Reduced MHC expression

MICRO (22 decks)

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