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IMM Exam 2 > Primary Immune Deficiency > Flashcards

Primary Immune Deficiency Flashcards

1
Q

definition of primary immune deficiency

A
  • genetic mutation that may occur at any phase of the immune response
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2
Q

primary immune deficiencies create undue susceptibility to

A
  • infection
  • autoimmunity
  • malignancy
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3
Q

inheritance of primary immune deficiencies

A
  • AR
  • AD
  • X-linked
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4
Q

most primary immune deficiencies are present in which stage of life?

A
  • childhood
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5
Q

importance of maternally transmitted IgG and primary immune deficiencies

A
  • can mask immunodeficiency up until about 6 months of age
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6
Q

majority of immune deficiencies are due to which cell?

A
  • B cells or humoral immunity
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7
Q

categories of immunodeficiency

A
  • humoral/antibody defects
  • cellular (mixed)
  • innate immunity
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8
Q

what kind of infections do you get with humoral/ antibody defects?

A
  • pyogenic infections with encapsulated bacteria
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9
Q

what diseases do you get with humoral/antibody defects?

A
  • recurrent otitis media
  • sinusitis
  • pneumonia
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10
Q

viral infection and humoral/antibody defects

A
  • frequent viral infections
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11
Q

diarrhea and humoral/antibody defects

A
  • chronic diarrhea
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12
Q

too frequent number of infections/antibiotics

A
  • > 4 courses of antibiotics per year in children
  • > 2 courses of antibiotics per year in adults
  • > 4 new ear infections in one year after age 4
  • pneumonia twice over any time
  • > 3 episodes of bacterial sinusitis in one year
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13
Q

Agamaglobulinemias caused by

A
  • defects in B cell development
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14
Q

Agamaglobulinemias and germinal center

A
  • germinal center formation is defective
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15
Q

Agamaglobulinemias and lymphoid tissues

A
  • underdevelopment of lymphoid tissues
  • lymph nodes
  • spleen
  • TONSILS AND ADENOIDS
  • Peyer’s patches
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16
Q

good physical sign for Agamaglobulinemias

A
  • no tonsils or adenoids
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17
Q

XLA accounts for what percentage of all Agamaglobulinemias

A
  • 85%
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18
Q

another name for XLA

A
  • bruton’s Agamaglobulinemias
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19
Q

XLA/Bruton’s caused by

A
  • failure of B cell maturation
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20
Q

XLA/Bruton’s or Bruton’s defect

A
  • B cell tyrosine kinase
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21
Q

populations that XLA/Bruton’s affects

A
  • boys

- lyonized females

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22
Q

role of B cell tyrosine kinase

A
  • required for transducing signals from pre-B cell receptor

- stimulates B cell maturation

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23
Q

IgG in B XLA

A
  • below 100 mg/dl
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24
Q

B cells in XLA

A

<2% of lymphocytes

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25
Q

T cells in XLA

A
  • normal cell number and function
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26
Q

Cause of Hyper IgM syndrome

A
  • defects in B cell isotype switching
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27
Q

number of B cells in hyper IgM syndrome

A
  • normal numbers
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28
Q

levels of antibodies in hyper IgM syndrome

A
  • elevated IgM levels

- low IgG, IgE, and IgA

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29
Q

frequency of IgA deficiency

A
  • most common primary immunodeficiency
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30
Q

IgA levels in IgA deficiency

A
  • <5-7mg/dl
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31
Q

symptomology of IgA deficiency

A
  • usually asymptomatic

- sometimes increased sinopulmonary infections, diarrhea, autoimmune disease

32
Q

giving IVIg to people with IgA deficiency?

A
  • bad idea!

- they have made antibodies against IgA

33
Q

frequency of common variable immune deficiency

A
  • second most frequent PID

- most symptomatic

34
Q

common variable immune deficiency most prevalent in

35
Q

common variable immune deficiency antibody levels

A
  • reduced IgG, IgA, and/or IgM
36
Q

common variable immune deficiency antibody response

A
  • absent or impaired antibody response to previous vaccination or infection
37
Q

way to check for common variable immune deficiency

A
  • check tetanus titer
38
Q

infections with common variable immune deficiency

A
  • sinusitis, pneumonia
  • life threatening infections possible
  • malignancies
39
Q

specific antibody deficiency infections

A
  • recurrent sinopulmonary infections
40
Q

specific antibody deficiency antibodies levels

A
  • normal IgG, IgA, IgM
41
Q

specific antibody deficiency B cell number and function

42
Q

specific antibody deficiency T cell number and function

43
Q

how to tell a specific antibody deficiency

A
  • impaired vaccine response (polysaccharide)

- impaired response to natural encapsulated bacteria

44
Q

encapsulated bacteria examplse

A
  • streptococcus pneumoniae (SEEN A LOT) and pyogenes
  • staph aureus
  • klebsiella
  • haemophilus influenzae
  • pseudomonas aeruginosa
  • nisseria meningitidis
  • cryptococcus neoformans

STATISTICALLY SOME KILLERS HAVE PRETTY NICE CAPSULES

45
Q

Transient hypogammaglobulinemia of infancy infections

A
  • recurrent sinopulmomary infections
46
Q

Transient hypogammaglobulinemia of infancy number of antibodies

A
  • slightly low IgG

- normal specific antibodies

47
Q

Transient hypogammaglobulinemia of infancy lymphocyte number and function

48
Q

Transient hypogammaglobulinemia of infancy cause

A
  • delay in maturation of T cell help for antibody production
49
Q

Transient hypogammaglobulinemia of infancy onset

A
  • around 6 months of age

- after maternal antibody has gone away

50
Q

Transient hypogammaglobulinemia of infancy resolves

51
Q

diagnostic evaluations for humoral immune system

A
  • CBC with diff
  • specific antibody titers
  • complement assays
  • lymphocyte markers (adjust for age)
52
Q

T cell dysfunctions most infected with

A
  • intracellular microorganisms
53
Q

Omenn syndrome caused by

A
  • mutations in RAG genes
54
Q

Omenn syndrome T cells

A
  • low to normal number
55
Q

Omenn syndrome onset

A
  • early onset < 3 months
56
Q

Omenn syndrome antibody markers

A
  • elevated IgE and Eosinophilia
57
Q

Omenn syndrome clinical signs

A
  • lymphadenopathy
  • hepatosplenomegaly
  • chronic persistent diarrhea
  • failure to thrive
58
Q

DiGeorge defect embryogenesis

A
  • defects in 3rd and 4th pharyngeal pouches
59
Q

wiskott aldrich syndrome clinical presentation

A
  • eczema
  • throbocytopenia with small platelets
  • immunodeficiency
60
Q

Wiskott Aldrich syndrome protein involved with

A
  • actin polymerization
61
Q

combined immunodeficiency humoral immunity

A
  • will also be impaired
62
Q

best diagnostic test for CGD diagnosis

A
  • flow cytometry
63
Q

leukocyte adhesion deficiency-1 and neutrophils

A
  • neutrophils cannot migrate toward inflammatory stimulate or adhere to vascular endothelium
64
Q

leukocyte adhesion deficiency-1 diagnosis suggestions

A
  • delayed umbilical cord separation
  • recurrent soft tissue infections
  • severe peridontal disease
  • no pus formation despite high white blood cell counts
65
Q

HyperIgE syndrome clinical presentation

A
  • severe eczema
  • retained primary teeth
  • recurrent candida
  • recurrent bone fractures
66
Q

HyperIgE syndrome antibody levels

A
  • very elevated IgE
    >2000
  • peripheral eosinophilia
67
Q

diagnostic tests for innate immunity

A
  • CBC with diff
  • neutrophil function
  • complement assay
68
Q

primary immune deficiencies that present at birth to 3 months

A
  • phagocytic cell defects
  • complement defects
  • DiGeorge
69
Q

primary immune deficiencies that present at 3-6 months

70
Q

primary immune deficiencies that present at 6-18 months

A
  • XLA

- transient hypogam

71
Q

primary immune deficiencies that present at 18 month-adulthood

A
  • CVI

- complement defects

72
Q

primary immune deficiency physical exam

A
  • growth measurements
  • inspection of tonsils
  • thrush
  • palpation of lymph nodes
  • organomegaly
  • skin lesions
73
Q

syndromes that you would treat with daily antibiotics

A
  • IgA deficiency
  • specific antibody deficiency
  • transient hypogammaglobulinemia
74
Q

syndromes that you would treat every 3-4 weeks with IVIG

A
  • XLA
  • hyper Igm syndrome
  • CVID
75
Q

best treatment for CGD

A
  • gamma interferon
76
Q

complement immunodeficiency treatment

A
  • immunizations with bacterial polysaccharide vaccines

IMM Exam 2 (15 decks)

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