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

Primary Immunodeficiency - Hogan Flashcards

(89 cards)

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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
Neutrophil disorders (CGD) leads to infections from (blank) producing organisms
catalase
26
Staph. aureus, Pseudomonas aeroginosa, Aspergillus fumigatus, candida, enterbacterieaie, and nocardia are (blank) producing organisms
catalase
27
Loss of phagocytic infections will usually show infections where?
bone, blood, and lungs
28
If a pt. comes in with a Hx of infection of numerous diff. types of organisms, what is your DDx?
phagocytic deficiency
29
Susceptiblity to NEISSERIA is caused by what?
Failure of the attack complex in complement
30
Absence of complement (blank) makes it difficult to respond to difficult bacterial infections
C3
31
T/F: fighting Neisseria requires a completely functional complement cascade
True
32
T/F: recurrent otitis media after middle school is a red flag. Why?
true. After middle school facial growth allows downhill drainage of sinuses
33
Otitis media, draining ears, sinusitis, and pulmonary infections are common in (blank ) cell deficiency which is (humoral/cell mediated)
B cell, humoral
34
Moraxella, H. influenzae, and Strep pneumoniae are common organisms infecting what age group?
kids
35
Odd organisms (pneumocystis carinii, invasive candida, systemic viral illness, or mycobacterail TB) can be associated with (T/B) cell problems
T cell
36
The AIDS epidemic allowed which bug to be better characterized?
pneumocystis carinii
37
When will you see disseminated TB in a child? What cell type is faulty?
countries that use the live vaccine; child will fail to produce Abs and will have a systemic reaction. T cells are faulty
38
T/F: T cell deficiencies can present with a child without lymph nodes
yes!
39
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?
T cell
40
T/F: B cell deficient patients get body wide severe rashes
FALSE: T cell
41
Why do T-cell def. pts fail to thrive?
all of their energy is going to constantly fighting infections
42
what must you do to blood when transfusion infants to prevent GvH since you don't know what their t-cells are like?
irradiate the blood
43
A Vaccine failure (besides BCG) is characterstic of (t/B) def?
B cell deficiency
44
Onset after 6 mos, recurrent virulent bacterial infections, allergies/autoimmune disease, and sinopulmonary infections are characteristic of (t/b) cell def?
B cell deficiency
45
T/F: B cell deficiency can present with or without failure to thrive
true
46
T/F: phagocytic deficiencies can range from mild to severe
true
47
Delayed separation of the umbilical cord is indicative of what type of deficiency?
phagocytic
48
severe infections (blood, bone, lung) skin infections, lymphadenitis, and abscesses are what type of deficiency?
phagocytic
49
What complement is deficient in recurrent bacterial infections?
C3
50
What complement components (besides the whole damn thing) are really important in Neisseria?
C5-8
51
If you suspect immunodef, what is the first lab you should order?
CBC with diff
52
Chediak-Higashi presents with (blank) granules
azurophilic
53
A bilobed neutrophil nucleus is telltale of (blank)
specific granule deficiency
54
Do you see increased or decreased neutrophils with LAD?
increased
55
What three things should you consider with neutropenia?
1. congenital absence 2. autoantibody 3. cyclical neutropenia
56
If WBC count is super high, what could it suggest besides an infection?
error in diapedesis and cellular adhesion
57
Low numbers of small platelets make you think of:
Wiskott-Aldrich syndrome (WAS)
58
Autoimmune hemolytic anemia and G6PD deficiencies are associated with what cell type?
RBCs
59
If you see severe lymphopenia, what should you consider?
SCID
60
What lab tests the function of the complement system? How do you interpret it?
CH50 and AH50; anything but 0 is good
61
T/F: sepsis with disseminated intravascular coagulation can deplete the complement system for 4-6 weeks
true
62
What complement proteins do AH50 and CH50 share?
C3 and C5-9
63
t/f: age makes a difference in interpreting quantitative Ig levels
true
64
Do you need to order specific subclasses of Ig's?
nope
65
What is an acceptable range for IgA serum levels?
Anything greater than zero
66
Why do you order albumin when determing B-cell function?
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
Nasal polyps in children indicate testing for what two disorders?
1. CF | 2. Immotile cilia syndrome
68
what are the three protein vaccines?
Diptheria, Tetanus, H flu | don't touch HAMsters
69
What is the polysaccharide vaccine?
Pneumovax
70
What do you order to test IgM function?
Isohemagluttinin levels
71
How long does it take to produce IgG after receiving a booster?
one month
72
If all Igs are decreased, what receptor should you start looking at?
CD receptor family
73
If CD19 is 0, what two emergencies come to mind?
XLA and SCID
74
What CD's are involved in T-cell disroders?
CD3, 4, 8, 19, 56
75
T/F: you should look at T, B, and NK cell markers to check for SCID
true
76
Mitogens like PHA, Con A, or pokeweed are used to test what?
to see if you can stimulate lymphocytes
77
What are the two either/or requirements for specific antigen testing?
Either one year of age and/or vaccinated against the bug
78
What does TREC analyze?
T cell excision circles to determine if T-cells are leaving the thymus
79
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?
diGeorge 22q11, via FISH; t-cell deficiency
80
The Sailboat sign on CXR indicates the presence of the:
thymus
81
If a kid doesnt have a thymus then he will have a (blank) cell deficiency
T-cell
82
CD11/18 flow cytometry can be used to determine problems with what cell?
phagocytes
83
What are the conditions to diagnose cyclical neutropenia?
3 week interval of symptoms with 6 day serial counts
84
What are some of the symptoms of cyclical neutropenia?
fever, gingivitis, oral ulcers--all from opportunistic infections
85
G6PD levels, MPO levels, bactericidal assays and IgE levels can be used to determine the function of what cell?
phagocytes
86
When looking at flow cytometry, what do you see with a normal PMA stimulation?
a right shift of the the peak
87
When looking at flow cytometry, what do you see with a Pho91-deficiency (X-linked)?
No shift or change of the peak
88
When looking at flow cytometry, what do you see with Pho47 deficient CGD (autosomal recesssive)?
a mild right shift and a wide broadening and shortening of the peak
89
When looking at flow cytometry, what do you see with an x-linked CGD carrier?
Two smaller peaks of comparable size, both right shifted.

Block 6 Week 1 Colt (11 decks)

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