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Immunology block 2 > Chapter 14 > Flashcards

Chapter 14 Flashcards

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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
Defect in C3 leads to?
Susceptibility to encapsulated bacteria
26
Defect in C5-C9?
Susceptibility to Neisseria because no MAC is formed
27
Defect in Factor D, properdin (factor P)?
Susceptible to Neisseria but no immune complex disease
28
Defect in factor I?
Similar effects to defect in C3. Susceptible to encapsulated bacteria
29
Defect in DAF, CD59?
Autoimmune like conditions like Paroxysmal nocturnal hemoglobinuria
30
C1INH deficiency?
Hereditary angioneurotic edema (HANE)
31
A deficiency in MBL will inhibit which complement pathway?
Lectin
32
What is the clinical presentation of an MBL deficiency?
Recurrent severe infections
33
Spontaneous activation of C1 is always occurring. If you have a deficiency in C1INH, what will be the result?
Systemic edema because of the anaphylatoxins produced from excessive C4bC2a C3 convertase of the classical pathway. Nothing was stopping C1
34
Syndrome that results from C1INH deficiency?
HANE
35
Patient has hemolytic anemia, red urine, and thrombosis. What disease do they have?
PNH
36
PNH comes with deficient DAF and CD59. Why are these proteins defective?
Because there is a genetic deficiency in glycophosphatidylinositol which is required for surface expression of CD59 and DAF
37
What do CD59 and DAF do?
Inhibit MAC formation on host cells
38
What is the treatment for PNH?
Allogenic bone marrow transplantation
39
Result in susceptibility to extracellular encapsulated bacteria
Antibody deficiencies
40
How do you treat antibody deficiencies?
Gamma globulin injection from healthy donors
41
Defect in Burton's tyrosine kinase which is involved in the signal transduction in B cells
X-linked agammaglobulinemia
42
Very few B cells develop in this disease
XLA
43
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)
Pre-B cell Receptor (lambda5) Deficiency
44
Defect in CD40 ligand on T-cells
X-linked hyper IgM Syndrome
45
Defect in AID (AID does class switching in Ig's)
X-linked hyper IgM Syndrome
46
Why is it called hyper IgM syndrome?
With deficient AID, you cannot class switch so you only have IgM
47
Would you rather suffer from an X-linked hyper IgM syndrome where there was a defect in CD40 ligand or a defect in AID?
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
Do patients with hyper IgM have germinal centers in their lymph nodes?
Apparently not!
49
Patients are susceptible to parasite pathogens. What deficiency do they have?
Selective IgA Deficiency
50
Susceptible to many viral and bacterial pathogens. Very rare
IgG1 deficiency
51
Susceptible to encapsulated bacteria. Most common in kids
IgG2 deficiency
52
Most common in adults?
IgG3 deficiency
53
Unknown significance?
IgG4 deficiency
54
Most common isotope of IgG circulating in blood in adults?
IgG1 (60-70%)
55
Most common immunodeficiency disorder?
CVID
56
A TAP transporter deficiency or a CD8 chain defect are both examples of what cellular deficiency?
CD8 T cell defects
57
Very low levels of MHC I and defective responses to intracellular pathogens are seen in this deficiency?
What is CD8 T cell deficiency
58
Bare lymphocyte syndrome?
MHC I deficiency
59
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?
He has a non-sense perforin mutation
60
SCID results from what deficiency?
CD4 T cell, because CD4 T cells are critical to both antibody mediated and cell mediated immune responses
61
Lack of expression of MHC II?
Bare lymphocyte syndrome
62
Inability of cell to deliver cytokines to other cells?
Wiskott-Aldrich
63
Results in toxic nucleotide metabolites that kills developing B and T cells
ADA
64
Impaired signaling in T cells?
Common gamma chain or Jak3 deficiency
65
Lack of CD4 or CD8 T cells; total lack of function
CD3 deficiency
66
Mis-sense mutations that result in partially activated RAG enzymes
Omenn syndrome
67
Result of Omenn syndrome
Absence of B cells; low numbers of oligoclonal auto reactive T cells
68
Absence of CD8 T cells but normal numbers of non-functional CD4 T cells. Results in SCID
ZAP-70 deficiency
69
Genetic deficiency of AIRE
APECED
70
Function of AIRE?
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
Genetic deficiency of FoxP3?
IPEX
72
Presentation of watery diarrhea, eczematous dermatitis, endocrinopathy. What disease?
IPEX
73
Immune cells fail to undergo apoptosis following an immune response. Causes overpopulation of secondary lymphoid tissue
ALPS
74
Results from mutation that prevents expression of Fas, Fas Ligand, Caspase 10
ALPS

Immunology block 2 (5 decks)

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