Ch8: Immunodeficiency and Transplantation Flashcards Preview

MICRO > Ch8: Immunodeficiency and Transplantation > Flashcards

Flashcards in Ch8: Immunodeficiency and Transplantation Deck (86):
1

What is primary immunodeficiency?

Due to genetic mutations and are inherited

2

Types of primary immunodeficiency?

Dominant
Recessive
X-linked

3

What is secondary immunodeficiency?

Acquired/Environmental

4

What can cause secondary immunodeficiency?

Irradation
Drug exposure
Infection

5

Classical complement pathway deficiency results in what?

Immune-complex disease

6

MBL pathway deficiency results in what?

Bacterial infections

7

Alternative pathway deficiency results in what?

Infection with pyogenic bacteria and neisseria

8

Problem with C3b deposition results in what?

Pyogenic bacteria and Neisseria (maybe immune-complex)

9

Problem with C5 through C9 result in what?

Deficiency with Neisseria species

10

Problem with DAF or CD59?

Auto-immune conditions

11

Problem with C1INH results in what?

Hereditary angioneurotic edema (HANE)

12

What is the genetic of C1INH disease?

Autosomal dominant

13

C1INH is a member of what family of molecules?

Serpin protease inhibitors

14

How does C1INH work?

Binds to C1r or C1s and is cleaved by it.

15

HANE results in what?

Swelling of face, larynx and abdomen

16

HANE is commonly seen when?

Trips to dentist

17

Leukocyte adhesion deficiency results in what?

No recruitment of phagocytes to infection site resulting in widespread infections

18

What is cause of chronic granulomatous disease?

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

19

What is G6PD deficiency?

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

20

What is myeloperoxidase deficiency?

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

21

What is Chediak-Higashi syndrome?

Defect in forming phagolysosome so results in persistent bacteria infections

22

LAD-1 deficiency is caused by what?

Mutations in Beta-2 integrins

23

LAD-2 deficiency is caused by what?

No sialyl lewis

24

Herpes simplex I is caused by what?

Defective antiviral immunity in CNS

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