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Lindsey's Blood and Lymph Unit III > Deficiency of Adaptive Immunity (Immune Deficiencies) > Flashcards

Deficiency of Adaptive Immunity (Immune Deficiencies) Flashcards

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1
Q

What is the incidence rate of X-linked (Bruton) Agammaglobulinemia?

A

0.4/100,000

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1
Q

Which cell induces class switching in the B cell?

A

Tfh

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1
Q

Name 2 advanced tests for phagocyte presence/function.

A
  1. assays for phagocytosis, chemotaxis 2. genetics
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3
Q

What is a secondary immunodeficiency?

A

a process outside of the immune system has caused the problem

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4
Q

What enzyme to SCID pts lack?

A

adenosine deaminase (ADA)

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4
Q

What is the syndrome called where pts have high IgM with low IgG and IgA?

A

X-linked hyperIgM syndrome

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4
Q

People with CVID are at increased risk for ____, ____, and ____.

A

lymphoma, enteropathy, autoimmunity

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5
Q

T-cell deficiencies are assoc. with intracellular pathogens, especially _____ and _____.

A

Candida albicans; Pnemocystis jirovecii

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6
Q

What is the defect in X-linked hyperIgM syndrome?

A

IgM-to-IgG switch mechanism

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6
Q

___ deficiencies are assoc. with high grade bacterial infections, such as Staph. aureus, H. influenzae, and Strep. pneumoniae.

A

B-cell

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7
Q

What is the syndrome where the thymus develops abnormally, resulting in absent T cells and normal B cells?

A

DiGeorge Syndrome

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8
Q

Name 2 initial tests for phagocyte presence/function.

A
  1. WBC count, diff, morph 2. NBT test, oxidative burst
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9
Q

B-cell deficiencies are assoc. with ____, such as Staph. aureus, H. influenzae, and Strep. pneumoniae.

A

high grade bacterial infections

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10
Q

How is AT inherited?

A

auto recessive

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11
Q

How is Wiskott-Aldrich syndrome inherited?

A

X-linked

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13
Q

What role does adenosine play in cellular development?

A

it accumulates in all cells, but impairs lymphocyte development in most severe

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14
Q

What is the cause of DiGeorge Syndrome?

A

large (45 gene) deletion on chromosome 22

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14
Q

What is the most common disease of immunodeficiency, and what is the incidence rate?

A

Selective IgA deficiency; 1/500

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15
Q

Name 4 initial tests for B cell presence/function.

A
  1. serum protein electrophoresis 2. quantitative IgG, A, M levels 3. specific Abs to prior immunizations 4. ABO isohemagglutinins
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17
Q

What is the life span of a pt with SCID?

A

1 year

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18
Q

Name 2 advanced tests for T cell presence/function.

A
  1. CD3, CD4, CD8 counts 2. mitogen responses, MLR, cytokine measurements
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18
Q

Name an advanced test for complement presence/function.

A
  1. individual complement component levels
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19
Q

AT patients have a very high rate of tumors because of?

A

defective DNA repair

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21
Q

What ligand does the Tfh cell interact with on the B cell?

A

CD40

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22
What is the most common form of SCID in Navajo children and what is the incidence rate?
defect in V(D)J recombination; 50/100,000
22
What are the s/s/findings in SCID?
lymphopenia of both T and B cells; absent thymic shadows; small tonsils; serum immunoglobulins are low
23
Primary immunodeficiencies affect ____ cells.
T or B, or both,
23
What is common variable immunodeficiency (CVID)?
B cells are difficult to trigger specific antibody; serum IgG low
24
What is telangiectasia?
dilated abnormal blood vessels
25
What is Transient Hypogammaglobinemia of Infancy?
slow IgG build up in affected children
26
Name 3 immunosuppressive viral illnesses.
1. measles 2. mono 3. cytomegalovirus (CMV)
27
What illnesses are seen in Bruton disease pts?
pneumonia, chronic diarrhea, enteroviruses (polio)
29
What is the main phenotype in CVID?
recurrent bacterial infections
30
What is the cause of AT?
IgA depressed; defective DNA repair; T and B cell deficiency
31
Name 2 initial tests for T cell presence/function.
1. skin test with recall Ag panel 2. total lymphocyte count
32
How does SCID occur?
block in the development of the lymphoid stem cell or its maturation
32
T-cell deficiencies are assoc. with \_\_\_\_, especially Candida albicans and Pnemocystis jirovecii.
intracellular pathogens
33
What is the incidence rate of DiGeorge Syndrome?
30/100,000
34
The stroma of the thymus comes from the \_\_\_\_\_\_.
endoderm and ectoderm of the 3rd and 4th pharyngeal pouches
35
What is a primary immunodeficiency?
disease w/ a genetic cause
36
How is SCID inherited?
X-linked in 50% of SCID-X1; auto recessive in the rest
37
Who gets Selective IgA deficiency?
it's familial; 10-15x more common in people with Celiac's Disease
38
What are the treatments of immunodeficiency?
1. isolation 2. prophylactic antibiotics 3. human immunoglobulin 4. transplantation
40
What does SCID stand for?
severe combined immunodeficiency disease
41
In CVID, serum IgG is low, at ____ or less.
0.5g/dL
42
What are the blood and marrow findings in X-linked (Bruton) Agammaglobulinemia?
pre-B cells in the bone marrow but no B cells or antibody
44
What is the treatment for CVID?
IVIG or SCIG
46
\_\_\_\_ deficiencies are assoc. with intracellular pathogens, especially Candida albicans and Pnemocystis jirovecii.
T-cell
47
The lymphoid part of the thymus comes from \_\_\_\_.
precursors in the bone marrow
48
What is the defect in X-linked (Bruton) Agammaglobulinemia?
btk gene, a protein tyrosine kinase, is defective
50
Why is DiGeorge related to calcium deficient convulsions?
parathyroid glands also derive from the pharyngeal pouches
50
When does Transient Hypogammaglobinemia of Infancy present, and how long does it last?
6 mos old; lasts 18 mos
51
B-cell deficiencies are assoc. with high grade bacterial infections, such as \_\_\_\_, \_\_\_\_\_, and \_\_\_\_\_.
Staph. aureus; H. influenzae; Strep. pneumoniae
52
Name 2 advanced tests for B cell presence/function.
1. Ab responses to novel Ags 2. sequencing of suspect genes
53
What are the s/s of Selective IgA deficiency?
usually asymptomatic, but can have diarrhea, sinopulmonary infections, or increased freq and severity of allergies
54
What is DiGeorge Syndrome?
the thymus develops abnormally, resulting in absent T cells and normal B cells
55
What is the defect in SCID-X1?
defective gene for gamma chain that forms part of the receptors for IL-2
57
What is Wiskott-Aldrich syndrome?
platelet and B cell deficiency, eczema, and bacterial infections
58
What is ataxia?
staggering
59
How do kids with Transient Hypogammaglobinemia of Infancy present?
recurrent and persistent gram-positive bacterial infections; chronic diarrhea
60
What is the most serious condition involving 2a immunodeficiency?
AIDS
61
The _____ of the thymus comes from precursors in the bone marrow.
lymphoid part
63
If there are normal T cells but low to absent B cells, there must be a devel. block between the ____ and the \_\_\_\_.
pre-B cell; B cell
64
Name 2 initial tests for complement presence/function.
1. CH50 2. assay for C1inh
65
What is AT?
sinus infections, pneumonia, ataxia, and telangiectasia
66
How does DiGeorge Syndrome affect the heart?
abnormal development of the great vessels
67
The ____ of the thymus comes from the endoderm and ectoderm of the 3rd and 4th pharyngeal pouches.
stroma

Lindsey's Blood and Lymph Unit III (10 decks)

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