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MICRO > Ch8: Immunodeficiency and Transplantation > Flashcards

Ch8: Immunodeficiency and Transplantation Flashcards

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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
Recurrent bacteria pneumonia is caused by what?
Defective innate immune response to pyogenic bacteria.
26
X-linked agammaglobulinemia (XLA) is a defect in what?
Btk that does intracellular signaling in B cell receptor
27
People with XLA can develop B cells how far?
Pre-B cell
28
Defects in ADA or PNP result in what?
No Pro or Pre B or T cells
29
Defects in RAG1 and RAG2 result in what?
No Pre B or T cells
30
Defect in Zap70 results in what?
No cytotoxic T cells
31
Defect in MHC class II results in what?
No CD4 T cells
32
IL-12 receptor deficiency results in what??
Recurrent intracellular bacteria like myobacterium
33
Which is worse, absence of a cytokine or a receptor?
Receptor because cytokines can be compensated by other cytokines
34
No IL-12 would mean what?
Can't activate NK Cells | Can't activate CTL hence Th1 cells
35
No IFN-gamma receptor would result in what?
Inability to clear intracellular bacteria because macrophages wouldn't respond to IFN-gamma
36
What cell signaling is interrupted with no IFN-gammareceptor?
Jak/STAT
37
Defect in IL-2 receptor gamma signaling chain affects what?
Affect signaling for a ton of cytokines
38
X-linked SCID is what?
No gamma chain on IL-2 receptor
39
Autosomal recessive SCID is what?
Jak3, ada, pnp
40
X-linked hyper IgM syndrome patients lack what?
CD40
41
What happens in x-linked hyper IgM?
No CD40 so B cells are not activated and continue to release IgM at high amounts
42
STAT3 mutation would result in what?
No Th17 cells
43
No IL-12R or IFNgamma-R's would result in what?
No Th1's
44
AID mutations result in what?
Hyper-IGM syndrome (autosomal)
45
B cell deficiencies have what abnormalities? | What takes advantage?
No germinal centers Reduced serum Ig levels Pyogenic bacterial infections
46
T cell deficiencies have what abnormalities? 3 | What takes advantage?
Reduced T cell zones Reduced DTH reactions Defective T cell proliferation Viruses
47
Innate immune deficiencies have what consequences? (2)
1. pyogenic bacteria | 2. Viruses
48
How long does it take for IgG to be made well enough by child?
1 year
49
HIV has what effect on immune?
No CD4 T helper
50
Irradiation and chemo have what effect on immune?
No progenitor lymphocytes
51
Immunosuppression for graft rejection and inflammatory diseases cause what?
Less lymphocytes
52
Bone marrow cancers have what effect on immune?
Less leukocyte development
53
Protein-calore malnutrition result in what?
Metabolic derangements that affect lymphocytes
54
Removal of spleen results in what?
Less phagocytosis of microbes
55
Two types of rejections?
Host vs graft | Graft vs host
56
Hyperacute rejection is the result of what?
Pre-formed antibodies
57
Acute rejection is the result of what?
Direct allorecognition by pre-formed T cells
58
Chronic rejection is the result of what?
Indirect allorecognition where a whole immune response is developed.
59
What is xenograft?
Donor and recipient different species
60
What is allograft?
Donor and recipient same species
61
What is syngraft?
Donor and recipient are twins
62
What is autograph?
Donor is recipient
63
Which are generally rejected of the types of grafts?
Xeno and ALlo
64
Rejection of organ is mediated by what? 2
Antibody | T cells
65
What is the best donor blood?
O-negative
66
What happens in hyperacute rejection of fetus?
Mother has antibodies against father's HLA/MHC in fetus
67
What is hyperacute transplant rejection?
Pre-formed antibodies bind to the transplant are complement cascade is activated which results in cell lysis through MAC formation
68
What is a microcytotoxicity test?
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
What is mixed lymphocyte reaction?
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
What is direct allorecognition due to?
Trying to match MHC before between donor and recipient
71
What happens in direct allorecognition?
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
How long til indirect allorecognition occurs?
Years
73
What is indirect allorecognition?
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
What specifically happens in both direct and indirect allorecognition?
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
Indirect allorecognition can induce what?
Antibodies
76
How to stop anti T cell antibodies? (2)?
1. Anti-lymphocyte serum | 2. Anti- T cell serum
77
Three main categories of immunosuppressive drugs?
1. Corticosteroids 2. Cytotoxic drugs 3. Microbial products
78
Corticosteroids have what main response?
Change gene expression and shut down inflammation
79
Cytotoxic drug treatment results in what?
Death of proliferating cells
80
Microbial products have what effect in immune?
INhibit T cell activation?
81
Three examples of microbial products?
1. Cyclosporin A 2. Tacrolimus 3. Rapamycin
82
Microbial products inhibit what?
T-cell receptor signaling
83
When does graft versus host disease occur?
Following allogenic or xenogeneic bone marrow transplant
84
GVHD leads to what?
Organ damage and death
85
What are immune privileged sites?
Sites that tolerate graft well without an immune response such as eyes, brain, testis, and bone.
86
What makes a site immune privileged?
Reduced MHC expression

MICRO (22 decks)

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