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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

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
Q

What is the most common form of SCID in Navajo children and what is the incidence rate?

A

defect in V(D)J recombination; 50/100,000

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22
Q

What are the s/s/findings in SCID?

A

lymphopenia of both T and B cells; absent thymic shadows; small tonsils; serum immunoglobulins are low

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23
Q

Primary immunodeficiencies affect ____ cells.

A

T or B, or both,

23
Q

What is common variable immunodeficiency (CVID)?

A

B cells are difficult to trigger specific antibody; serum IgG low

24
Q

What is telangiectasia?

A

dilated abnormal blood vessels

25
Q

What is Transient Hypogammaglobinemia of Infancy?

A

slow IgG build up in affected children

26
Q

Name 3 immunosuppressive viral illnesses.

A
  1. measles 2. mono 3. cytomegalovirus (CMV)
27
Q

What illnesses are seen in Bruton disease pts?

A

pneumonia, chronic diarrhea, enteroviruses (polio)

29
Q

What is the main phenotype in CVID?

A

recurrent bacterial infections

30
Q

What is the cause of AT?

A

IgA depressed; defective DNA repair; T and B cell deficiency

31
Q

Name 2 initial tests for T cell presence/function.

A
  1. skin test with recall Ag panel 2. total lymphocyte count
32
Q

How does SCID occur?

A

block in the development of the lymphoid stem cell or its maturation

32
Q

T-cell deficiencies are assoc. with ____, especially Candida albicans and Pnemocystis jirovecii.

A

intracellular pathogens

33
Q

What is the incidence rate of DiGeorge Syndrome?

A

30/100,000

34
Q

The stroma of the thymus comes from the ______.

A

endoderm and ectoderm of the 3rd and 4th pharyngeal pouches

35
Q

What is a primary immunodeficiency?

A

disease w/ a genetic cause

36
Q

How is SCID inherited?

A

X-linked in 50% of SCID-X1; auto recessive in the rest

37
Q

Who gets Selective IgA deficiency?

A

it’s familial; 10-15x more common in people with Celiac’s Disease

38
Q

What are the treatments of immunodeficiency?

A
  1. isolation 2. prophylactic antibiotics 3. human immunoglobulin 4. transplantation
40
Q

What does SCID stand for?

A

severe combined immunodeficiency disease

41
Q

In CVID, serum IgG is low, at ____ or less.

A

0.5g/dL

42
Q

What are the blood and marrow findings in X-linked (Bruton) Agammaglobulinemia?

A

pre-B cells in the bone marrow but no B cells or antibody

44
Q

What is the treatment for CVID?

A

IVIG or SCIG

46
Q

____ deficiencies are assoc. with intracellular pathogens, especially Candida albicans and Pnemocystis jirovecii.

A

T-cell

47
Q

The lymphoid part of the thymus comes from ____.

A

precursors in the bone marrow

48
Q

What is the defect in X-linked (Bruton) Agammaglobulinemia?

A

btk gene, a protein tyrosine kinase, is defective

50
Q

Why is DiGeorge related to calcium deficient convulsions?

A

parathyroid glands also derive from the pharyngeal pouches

50
Q

When does Transient Hypogammaglobinemia of Infancy present, and how long does it last?

A

6 mos old; lasts 18 mos

51
Q

B-cell deficiencies are assoc. with high grade bacterial infections, such as ____, _____, and _____.

A

Staph. aureus; H. influenzae; Strep. pneumoniae

52
Q

Name 2 advanced tests for B cell presence/function.

A
  1. Ab responses to novel Ags 2. sequencing of suspect genes
53
Q

What are the s/s of Selective IgA deficiency?

A

usually asymptomatic, but can have diarrhea, sinopulmonary infections, or increased freq and severity of allergies

54
Q

What is DiGeorge Syndrome?

A

the thymus develops abnormally, resulting in absent T cells and normal B cells

55
Q

What is the defect in SCID-X1?

A

defective gene for gamma chain that forms part of the receptors for IL-2

57
Q

What is Wiskott-Aldrich syndrome?

A

platelet and B cell deficiency, eczema, and bacterial infections

58
Q

What is ataxia?

A

staggering

59
Q

How do kids with Transient Hypogammaglobinemia of Infancy present?

A

recurrent and persistent gram-positive bacterial infections; chronic diarrhea

60
Q

What is the most serious condition involving 2a immunodeficiency?

A

AIDS

61
Q

The _____ of the thymus comes from precursors in the bone marrow.

A

lymphoid part

63
Q

If there are normal T cells but low to absent B cells, there must be a devel. block between the ____ and the ____.

A

pre-B cell; B cell

64
Q

Name 2 initial tests for complement presence/function.

A
  1. CH50 2. assay for C1inh
65
Q

What is AT?

A

sinus infections, pneumonia, ataxia, and telangiectasia

66
Q

How does DiGeorge Syndrome affect the heart?

A

abnormal development of the great vessels

67
Q

The ____ of the thymus comes from the endoderm and ectoderm of the 3rd and 4th pharyngeal pouches.

A

stroma

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

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