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Immunology block 2 > Chapter 14 > Flashcards

Chapter 14 Flashcards

1
Q

Inherited disorder in which patients have elevated susceptibility to encapsulated bacterial pathogens (Strep pneumonia, Haemophilus influenzae)

A

Asplenia

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2
Q

Patients with asplenia are susceptible to encapsulated bacterial pathogens. What complication from these pathogens are they especially susceptible to?

A

Septic infections

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3
Q

Wasn’t really defined, but use common sense. What is asplenia?

A

Dysfunctional spleen

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4
Q

You have a patient that is constantly getting pneumonia. When he gets pneumonia, he always qualifies for SIRS (using the 4 parameters). He was in a car accident 10 years ago with trauma to his lower left abdominal quadrant. Possible diagnosis?

A

Aslpenia

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5
Q

What does the myeloperoxidase enzyme do?

A

Catalyzes production of hypochlorous acid; major component in killing of phagocytosed bacteria

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6
Q

Where does MPO reside?

A

Neutrophil granules and macrophage lysosomes

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7
Q

Neutrophil count of less than 500 cells/microL

A

Neutropenia

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8
Q

3 common forms of neutropenia?

A
  1. Severe congenital neutropenia (Kostman syndrome)
  2. Cyclic neutropenia
  3. Benign chronic neutropenia
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9
Q

Do you have to know the differences in the different types of neutropenia?

A

Not for this test

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10
Q

What are some primary immunodeficiencies that have associated neutropenia?

A
  1. X-linked hyper-IgM syndrome
  2. X-linked agammaglobulinemia (XLA)
  3. WHIM syndrome
  4. Griselli syndrome
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11
Q

Diagnosis for NK cell deficiency?

A

Flow cytometry for NK cells and NKT cells

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12
Q

Increased risk and severity of viral infections?

A

NK deficiency

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13
Q

Varicella, herpes, Epstein-Barr, mycobacterium avium, trichophyton… are all viruses/diseases that are common in people with what deficiency?

A

NK cell

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14
Q

3 ways a genetic deficiency can cause NK cell deficiency

A
  1. Defective formation of cytoplasmic granules
  2. Defective perforin
  3. Defects in development of bone marrow
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15
Q

Genetic defect in a protein required for NFkB activity

A

NEMO deficiency

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16
Q

What is NFkB?

A

Important transcription factor for physical development and innate immunity

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17
Q

What innate immune system molecule activates NFkB?

A

TLR’s

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18
Q

NEMO is aka?

A

X-linked hypohydrotic ectodermal dysplasia and immunodeficiency

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19
Q

When patients have recurrent bacterial and viral infections (mycobacterium avium), what do they probably have?

A

Immunodeficiency

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20
Q

How do you treat immunodeficiency?

A

Biweekly injections of gamma globulin from healthy donor; bone marrow transplant

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21
Q

Promising looking new treatment for immunodeficiency?

A

Stem cells from umbilical cords

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22
Q

Usual result of deficiency of complement protein?

A

Susceptibility to extracellular bacteria

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23
Q

Deficiency in complement regulatory proteins leads to?

A

Depletion of C3, therefore susceptibility to encapsulated bacteria. Also can lead to auto-immune like disease where complement destroys RBC’s

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24
Q

Defect in C1, C2, C4 leads to?

A

Immune complex disease

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25
Q

Defect in C3 leads to?

A

Susceptibility to encapsulated bacteria

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26
Q

Defect in C5-C9?

A

Susceptibility to Neisseria because no MAC is formed

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27
Q

Defect in Factor D, properdin (factor P)?

A

Susceptible to Neisseria but no immune complex disease

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28
Q

Defect in factor I?

A

Similar effects to defect in C3. Susceptible to encapsulated bacteria

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29
Q

Defect in DAF, CD59?

A

Autoimmune like conditions like Paroxysmal nocturnal hemoglobinuria

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30
Q

C1INH deficiency?

A

Hereditary angioneurotic edema (HANE)

31
Q

A deficiency in MBL will inhibit which complement pathway?

A

Lectin

32
Q

What is the clinical presentation of an MBL deficiency?

A

Recurrent severe infections

33
Q

Spontaneous activation of C1 is always occurring. If you have a deficiency in C1INH, what will be the result?

A

Systemic edema because of the anaphylatoxins produced from excessive C4bC2a C3 convertase of the classical pathway. Nothing was stopping C1

34
Q

Syndrome that results from C1INH deficiency?

A

HANE

35
Q

Patient has hemolytic anemia, red urine, and thrombosis. What disease do they have?

A

PNH

36
Q

PNH comes with deficient DAF and CD59. Why are these proteins defective?

A

Because there is a genetic deficiency in glycophosphatidylinositol which is required for surface expression of CD59 and DAF

37
Q

What do CD59 and DAF do?

A

Inhibit MAC formation on host cells

38
Q

What is the treatment for PNH?

A

Allogenic bone marrow transplantation

39
Q

Result in susceptibility to extracellular encapsulated bacteria

A

Antibody deficiencies

40
Q

How do you treat antibody deficiencies?

A

Gamma globulin injection from healthy donors

41
Q

Defect in Burton’s tyrosine kinase which is involved in the signal transduction in B cells

A

X-linked agammaglobulinemia

42
Q

Very few B cells develop in this disease

A

XLA

43
Q

This disease is from a mutation in lambda5 (a component of the surrogate light chain that pairs with the mu heavy chain during somatic recombination of light chain genes)

A

Pre-B cell Receptor (lambda5) Deficiency

44
Q

Defect in CD40 ligand on T-cells

A

X-linked hyper IgM Syndrome

45
Q

Defect in AID (AID does class switching in Ig’s)

A

X-linked hyper IgM Syndrome

46
Q

Why is it called hyper IgM syndrome?

A

With deficient AID, you cannot class switch so you only have IgM

47
Q

Would you rather suffer from an X-linked hyper IgM syndrome where there was a defect in CD40 ligand or a defect in AID?

A

One with a defect in AID because at least you would have IgM. Without CD40 ligand you would not activate any B cells thus you wouldn’t have any Ig’s

48
Q

Do patients with hyper IgM have germinal centers in their lymph nodes?

A

Apparently not!

49
Q

Patients are susceptible to parasite pathogens. What deficiency do they have?

A

Selective IgA Deficiency

50
Q

Susceptible to many viral and bacterial pathogens. Very rare

A

IgG1 deficiency

51
Q

Susceptible to encapsulated bacteria. Most common in kids

A

IgG2 deficiency

52
Q

Most common in adults?

A

IgG3 deficiency

53
Q

Unknown significance?

A

IgG4 deficiency

54
Q

Most common isotope of IgG circulating in blood in adults?

A

IgG1 (60-70%)

55
Q

Most common immunodeficiency disorder?

A

CVID

56
Q

A TAP transporter deficiency or a CD8 chain defect are both examples of what cellular deficiency?

A

CD8 T cell defects

57
Q

Very low levels of MHC I and defective responses to intracellular pathogens are seen in this deficiency?

A

What is CD8 T cell deficiency

58
Q

Bare lymphocyte syndrome?

A

MHC I deficiency

59
Q

A patient has normal levels of CD8 T cells (CTL’s), but he is constantly susceptible to intracellular infections. What do you think may be the problem?

A

He has a non-sense perforin mutation

60
Q

SCID results from what deficiency?

A

CD4 T cell, because CD4 T cells are critical to both antibody mediated and cell mediated immune responses

61
Q

Lack of expression of MHC II?

A

Bare lymphocyte syndrome

62
Q

Inability of cell to deliver cytokines to other cells?

A

Wiskott-Aldrich

63
Q

Results in toxic nucleotide metabolites that kills developing B and T cells

A

ADA

64
Q

Impaired signaling in T cells?

A

Common gamma chain or Jak3 deficiency

65
Q

Lack of CD4 or CD8 T cells; total lack of function

A

CD3 deficiency

66
Q

Mis-sense mutations that result in partially activated RAG enzymes

A

Omenn syndrome

67
Q

Result of Omenn syndrome

A

Absence of B cells; low numbers of oligoclonal auto reactive T cells

68
Q

Absence of CD8 T cells but normal numbers of non-functional CD4 T cells. Results in SCID

A

ZAP-70 deficiency

69
Q

Genetic deficiency of AIRE

A

APECED

70
Q

Function of AIRE?

A

Transcription factor that regulates expression of the self-peptides that are presented to developing thymocytes in the thymus. Defective AIRE means no negative selection

71
Q

Genetic deficiency of FoxP3?

A

IPEX

72
Q

Presentation of watery diarrhea, eczematous dermatitis, endocrinopathy. What disease?

A

IPEX

73
Q

Immune cells fail to undergo apoptosis following an immune response. Causes overpopulation of secondary lymphoid tissue

A

ALPS

74
Q

Results from mutation that prevents expression of Fas, Fas Ligand, Caspase 10

A

ALPS

Immunology block 2 (5 decks)

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