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Immunology II > primary immunodeficiency > Flashcards

primary immunodeficiency Flashcards

1
Q

primary immunodeficiencies

A
  • genetic mutation that may occur at any phase of the immune response
  • can lead to autoimmunity and malignancy
  • inheritance does play a rule
  • most present in childhood
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2
Q

maternally transmitted IgG can/cannot mask immunodeficiency up until about 6 months of age

A

can

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3
Q

agammaglobulinemia

A
  • caused by defects in B cell development
  • germinal center formation in these patients is defective (no peyers patch, lymph nodes, spleen, tonsils)
  • defect in b cell tyrosine kinase pre B cells cannot mature
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4
Q

t/f most primary immunodeficiencies are due to antibody deficiencies

A

true

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5
Q

t/f agammaglobulinemas are x-linked and autosomal recessive

A

true

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6
Q

x-linked agammaglobulinemias account for _____ % of agammaglobulinemias

A

85

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7
Q

agammaglobilnemia is also known as

A

Brutons

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8
Q

b cell tyrosine kinase

A

required for transducing signals from the pre-B cell receptor downstream to signal B cell maturation

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9
Q

x-linked agammaglobulinemia has approximately ____ % with positive family history

A

50%

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10
Q

which Ig is decreased in x-linked agammaglobulinemia

A

IgG , < 100 mg/dl

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11
Q

t/f people with agammaglobulinemia have normal t cell number and function

A

true

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12
Q

hyper-IgM syndrome

A

defects in B cell isotype switching collectively lead to a group of disorders called Hyper-IgM syndrome

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13
Q

t/f people with hyper-IgM syndrome express normal numbers of B cells but express low levels of IgM and elevated levels of IgG, IgE, and IgA

A

false
people with hyper-IgM syndrome express normal numbers of B cells but express high levels of IgM and low levels of IgG, IgE, and IgA

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14
Q

IgA deficiency

A
  • most common primary immunodeficiency
  • usually asymptomatic
  • less than 5-7 mg/dl
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15
Q

Common Variable Immunodeficiency (CVID)

A
  • second most frequent primary immunodeficient disease in humans
  • most prevalent PID in adults
  • recurrent infections
  • absent or impaired specific antibody responses
  • reduced serum of IgG IgA, and or IgM
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16
Q

specific antibody deficiency

A
  • recurrent sinoulmonary deficiency
  • normal IgG, IgA, and IgM
  • normal B cell number and normal T cell number and function
  • impaired vaccine response to polysaccharides
  • impaired antibody response to natural infection with encapsulated bacteria
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17
Q

encapsulated organisms

A 
Some Killers Have Pretty Nice Capsules
streptococcus pneumoniae and pyogens 
Staph aureus 
Klebsiella 
Haemophilus influenzae
Pseudomonas
Neisseria 
Cryptococcus
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18
Q

Transient Hypogammaglobulinemia of Infancy

A
  • recurrent sinopulmonary infections
  • low IgG but NORMAL specific antibodies
  • normal lymphocyte number and function
  • onset 6 months resolved by age 4
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19
Q

Transient Hypogammaglobulinemia of Infancy onset at _____ months of age and resolve by age _____

A

6; 4

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20
Q

two ways to check specific antibody production

A
  1. protein constituents (diphtheria and tetanus)

2. polysaccharides (isohemagglutinins) need to check blood type first

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21
Q

t-cell dysfunction clinical characteristics

A

infections with intracellular microorganisms

  • failure to thrive (cannot grow)
  • anergy
  • increased B-cell malignancies
  • thrush
  • eczema
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22
Q

omenn syndrome

A
  • leaky SCID
  • hypomorphic mutations usually in RAG genes
  • low to normal number of t -cells
  • exudative erythroderma
  • lymphadenopathy
  • hepatosplenomegaly
  • failure to thrive
  • elevated IgE
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23
Q

SCID x-linked

A

can affect t cell development

24
Q

DiGeorge Syndrome

A

defect in embryogenesis 3rd and 4th pharyngeal pouches

  • most have chromosome 22 deletino
  • clinical features: dysmorphic facies, hypocalcemia, congenital heart disease
  • depressed T cell immunity
  • partial (thymic hypoplasia) versus complete (thymic aplasia)
25
Q

wiskott aldrich syndrome

A
  • patients with eczema and thrombocytopenia with small platelets (MPV mean platelet volume)
  • involved in actin polymerization
26
Q

T cell mediated immunity tests

A
  1. lymphocyte markers (CBC with diff)
  2. Lymphocyte mitogen proliferation
  3. antigen reactivity
27
Q

chronic granulomatous disease

A

recurrent bacterial infection with catalase positive organisms
- phagocytic cells can ingest but not digest

28
Q

leukocyte adhesion deficiency 1

A
  • absent beta subunit (CD18)
  • neutrophils cannot migrate toward inflammatory stimuli or adhere to vascular endothelium
  • recurrent infections
  • delayed umbilical cord separation
  • severe periodontal disease
  • no pus formation despite high white blood cell counts
29
Q

hyper IgE syndrome

A
  • Job syndrome
  • severe ecxema
  • elevated IgE
  • retained primary teeth
  • recurrent candida and bone fractures
  • peripheral eosinophilia
30
Q

defects in C1 esterase inhibitor

A

hereditary angioedema
massive swelling
no itching or associated hives

31
Q

diagnostic tests in the diagnosis of defects in innate immunity

A
  1. CBC
  2. NEUTROPHIL FUNCTINO TEST
  3. ASSASYS FOR COMPLEMENT
32
Q

phagocytic cell defect, complement defects, and digeorges syndrome usually develop from

A

birth to 3 months

33
Q

3-6 months immunodeficient disease

A

SCID

6-18 months

34
Q

x-linked agammaglobulinemia is usually seen in

A

6-18 months

35
Q

18 months of age _____ and _____ are the immunodeficient diseases of concern

A

common variable immunodeficiency

complement defects

36
Q

humoral /antibody deficiency treatments

A
  1. avoidance of high rate exposure
  2. antibiotic therapy
    - IV or SQ IgG
37
Q

combined immunodeficiency treatment

A
  1. stem cell transplantation
  2. enzyme replacement
  3. thymic transplant
  4. gene therapy
  5. avoidance of live viral vaccines
38
Q

defects of phagocytic cells treatment

A
  1. prophylactic antibodies
  2. avoidance of live viral vaccines
  3. gamman interferon in chronic granulomatous disease
  4. bone marrow transplantation in some patients
39
Q

complement immunodeficiency treatment

A

symptomatic care
frequent use of antibiotics
immunizations with bacterial polysaccharide vaccines

40
Q

prevnar versus pneumovax

A

prevnar- conjugated pneumococcal vaccine more efficient vaccine

41
Q

t/f lack of t cells will result in combined immunodeficiencies

A

true

42
Q

t-cell dysfunction leads to issues with infections with intracellular/extracellular microorganisms

A

intracellular

43
Q

CH50 assay

A

classical pathway

44
Q

AH50

A

alternative pathway

45
Q

MBL

A

lectin

46
Q

c3 deficiency

A

more likely to have infection due to encapsulated bacteria

47
Q

C5 deficiency

A

increased risk of infection by neisserial infections

48
Q

IVIG

A

every three to four weeks

49
Q

SQ IgG

A

weekly

50
Q

thymic transplant

A

digeroges syndrome

51
Q

defects of phagocytic cells treatment

A

prophylactic antibiotics

52
Q

diphtheria and tetanus are polysaccharide/protein vaccines?

A

protein

53
Q

t cell/cellular immunodeficiencies are also called _______ because T cells are important in guiding the function of other immune cells

A

combined immunodeficiencies

54
Q

t cell/cellular immunodeficiencies are at greater risks for _____ organisms

A

opportunistic

55
Q

opportunistic organisms include

A

candida, PCP, and CMV

Immunology II (11 decks)

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