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Block 6 Week 1 Colt > Primary Immunodeficiency - Hogan > Flashcards

Primary Immunodeficiency - Hogan Flashcards

1
Q

What is the rate of primary immunodeficiency in the population?

A

1:500

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2
Q

What is the most common form of 1ry immunodef?

A

Antibody defectsw (b cell defects)

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3
Q

What is the 2nd most common form of 1ry immunodef?

A

T-cell defects, both combined with b-cell defects (20%) or standalone (10%)

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4
Q

T/F: Phagocytic disorders as a whole have a more homogenous presentation

A

True

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5
Q

What percent of 1ry immunodef makes up complement disorders?

A

2%

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6
Q

T/F: innate immune function is fully available at birth

A

True

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7
Q

Describe the migration of leukocyte development prior to birth?

A

From liver/spleen to bone marrow

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8
Q

T/F: Phagocyte functionality is complete in neonates

A

True

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9
Q

T/F: Complement function is available to neonates

A

True

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10
Q

by what method is complement available to babies?

A

maternal transfer of IgG

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11
Q

At what age will self production of complement begin?

A

6 months

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12
Q

T/F: NK function is available in neonates

A

True

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13
Q

T/F: T cell function is complete at birth

A

True

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14
Q

If T cells fail to develop, (blank) cell functionality will suffer

A

B-cell

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15
Q

(blank) cell maturation is required to complete B cell maturation to functional antibody function

A

T cell

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16
Q

How is maternal IgG given to the baby in utero?

A

Transplacentally

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17
Q

What Ig do you assay for infection in neonates? Why?

A

IgM; its the only Ab that is readily made at birth

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18
Q

At what age do you really expect any IgA to be seen?

A

2 years

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19
Q

autoimmune problems in the patient/family suggest problems with (blank) cell maturation/tolerance at a genetic level

A

B cell

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20
Q

T/F: Immunodeficiency always develops after autoimmunity

A

FALSE: immunodef can develop BEFORE OR AFTER autoimmunity

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21
Q

Is autoimmunity cell-mediated or humoral?

A

humoral

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22
Q

What causes a majority of autoimmune issues?

A

Failure of B cell maturation/isotope switching/tolerance for self

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23
Q

Severe infections, lymphadenitis, osteomyelitis, pneumonia, and sepsis are signs of a def. in (innate/adaptive) immunity?

A

innate

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24
Q

phagocytes are (discriminatory/nondiscriminatory) in types of foreign organisms attacked

A

non discriminatory:
Gram pos
Gram neg
yeast/fungi

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25
Q

Neutrophil disorders (CGD) leads to infections from (blank) producing organisms

A

catalase

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26
Q

Staph. aureus, Pseudomonas aeroginosa, Aspergillus fumigatus, candida, enterbacterieaie, and nocardia are (blank) producing organisms

A

catalase

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27
Q

Loss of phagocytic infections will usually show infections where?

A

bone, blood, and lungs

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28
Q

If a pt. comes in with a Hx of infection of numerous diff. types of organisms, what is your DDx?

A

phagocytic deficiency

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29
Q

Susceptiblity to NEISSERIA is caused by what?

A

Failure of the attack complex in complement

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30
Q

Absence of complement (blank) makes it difficult to respond to difficult bacterial infections

A

C3

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31
Q

T/F: fighting Neisseria requires a completely functional complement cascade

A

True

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32
Q

T/F: recurrent otitis media after middle school is a red flag. Why?

A

true. After middle school facial growth allows downhill drainage of sinuses

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33
Q

Otitis media, draining ears, sinusitis, and pulmonary infections are common in (blank ) cell deficiency which is (humoral/cell mediated)

A

B cell, humoral

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34
Q

Moraxella, H. influenzae, and Strep pneumoniae are common organisms infecting what age group?

A

kids

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35
Q

Odd organisms (pneumocystis carinii, invasive candida, systemic viral illness, or mycobacterail TB) can be associated with (T/B) cell problems

A

T cell

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36
Q

The AIDS epidemic allowed which bug to be better characterized?

A

pneumocystis carinii

37
Q

When will you see disseminated TB in a child? What cell type is faulty?

A

countries that use the live vaccine; child will fail to produce Abs and will have a systemic reaction. T cells are faulty

38
Q

T/F: T cell deficiencies can present with a child without lymph nodes

39
Q
  1. Family Hx
  2. Onset before 6 mos.
  3. Opportunistic infections
  4. Cutaneous lesions
  5. No lymph nodes
  6. increased chance of cancer
  7. failure to thrive
  8. GvH after transfusion or birth
  9. Severe fungal/viral infection
  10. Fatality after TB BCG vaccine
  11. Diarrhea BEFORE infection
  12. Hepatosplenomegaly
    These are all characteristics of (blank) cell deficiency?
40
Q

T/F: B cell deficient patients get body wide severe rashes

A

FALSE: T cell

41
Q

Why do T-cell def. pts fail to thrive?

A

all of their energy is going to constantly fighting infections

42
Q

what must you do to blood when transfusion infants to prevent GvH since you don’t know what their t-cells are like?

A

irradiate the blood

43
Q

A Vaccine failure (besides BCG) is characterstic of (t/B) def?

A

B cell deficiency

44
Q

Onset after 6 mos, recurrent virulent bacterial infections, allergies/autoimmune disease, and sinopulmonary infections are characteristic of (t/b) cell def?

A

B cell deficiency

45
Q

T/F: B cell deficiency can present with or without failure to thrive

46
Q

T/F: phagocytic deficiencies can range from mild to severe

47
Q

Delayed separation of the umbilical cord is indicative of what type of deficiency?

A

phagocytic

48
Q

severe infections (blood, bone, lung) skin infections, lymphadenitis, and abscesses are what type of deficiency?

A

phagocytic

49
Q

What complement is deficient in recurrent bacterial infections?

50
Q

What complement components (besides the whole damn thing) are really important in Neisseria?

51
Q

If you suspect immunodef, what is the first lab you should order?

A

CBC with diff

52
Q

Chediak-Higashi presents with (blank) granules

A

azurophilic

53
Q

A bilobed neutrophil nucleus is telltale of (blank)

A

specific granule deficiency

54
Q

Do you see increased or decreased neutrophils with LAD?

55
Q

What three things should you consider with neutropenia?

A
  1. congenital absence
  2. autoantibody
  3. cyclical neutropenia
56
Q

If WBC count is super high, what could it suggest besides an infection?

A

error in diapedesis and cellular adhesion

57
Q

Low numbers of small platelets make you think of:

A

Wiskott-Aldrich syndrome (WAS)

58
Q

Autoimmune hemolytic anemia and G6PD deficiencies are associated with what cell type?

59
Q

If you see severe lymphopenia, what should you consider?

60
Q

What lab tests the function of the complement system? How do you interpret it?

A

CH50 and AH50; anything but 0 is good

61
Q

T/F: sepsis with disseminated intravascular coagulation can deplete the complement system for 4-6 weeks

62
Q

What complement proteins do AH50 and CH50 share?

A

C3 and C5-9

63
Q

t/f: age makes a difference in interpreting quantitative Ig levels

64
Q

Do you need to order specific subclasses of Ig’s?

65
Q

What is an acceptable range for IgA serum levels?

A

Anything greater than zero

66
Q

Why do you order albumin when determing B-cell function?

A

Determine if IgG loss is secondary; IgG is processed very similarly to albumin, so if albumin is low, IgG is likely being lost by that same mechanism (2ry immune defect then)

67
Q

Nasal polyps in children indicate testing for what two disorders?

A
  1. CF

2. Immotile cilia syndrome

68
Q

what are the three protein vaccines?

A

Diptheria, Tetanus, H flu

don’t touch HAMsters

69
Q

What is the polysaccharide vaccine?

70
Q

What do you order to test IgM function?

A

Isohemagluttinin levels

71
Q

How long does it take to produce IgG after receiving a booster?

72
Q

If all Igs are decreased, what receptor should you start looking at?

A

CD receptor family

73
Q

If CD19 is 0, what two emergencies come to mind?

A

XLA and SCID

74
Q

What CD’s are involved in T-cell disroders?

A

CD3, 4, 8, 19, 56

75
Q

T/F: you should look at T, B, and NK cell markers to check for SCID

76
Q

Mitogens like PHA, Con A, or pokeweed are used to test what?

A

to see if you can stimulate lymphocytes

77
Q

What are the two either/or requirements for specific antigen testing?

A

Either one year of age and/or vaccinated against the bug

78
Q

What does TREC analyze?

A

T cell excision circles to determine if T-cells are leaving the thymus

79
Q

If your pt has a facial anomaly, cardiac defect, low calcium, and is seizing, what disorder does he likely have? How do you test for it? What cell type does he have problems with?

A

diGeorge 22q11, via FISH; t-cell deficiency

80
Q

The Sailboat sign on CXR indicates the presence of the:

81
Q

If a kid doesnt have a thymus then he will have a (blank) cell deficiency

82
Q

CD11/18 flow cytometry can be used to determine problems with what cell?

A

phagocytes

83
Q

What are the conditions to diagnose cyclical neutropenia?

A

3 week interval of symptoms with 6 day serial counts

84
Q

What are some of the symptoms of cyclical neutropenia?

A

fever, gingivitis, oral ulcers–all from opportunistic infections

85
Q

G6PD levels, MPO levels, bactericidal assays and IgE levels can be used to determine the function of what cell?

A

phagocytes

86
Q

When looking at flow cytometry, what do you see with a normal PMA stimulation?

A

a right shift of the the peak

87
Q

When looking at flow cytometry, what do you see with a Pho91-deficiency (X-linked)?

A

No shift or change of the peak

88
Q

When looking at flow cytometry, what do you see with Pho47 deficient CGD (autosomal recesssive)?

A

a mild right shift and a wide broadening and shortening of the peak

89
Q

When looking at flow cytometry, what do you see with an x-linked CGD carrier?

A

Two smaller peaks of comparable size, both right shifted.

Block 6 Week 1 Colt (11 decks)

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