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Immunodeficiencies Flashcards

1
Q

What are the differences between primary and secondary immunodeficiencies?

A

Primary are genetic diseases that cause immunodeficienies
Secondary are usually caused my something else like malnutrition, renal disease, transplants, diabetes etc. mostly preventable

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2
Q

Main cells that function in innate immunity?

4

A
  1. complements
  2. macrophages
  3. neutrophils
  4. NK cells
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3
Q

Main cells that funciton in acquired immunity?

4

A

B cells
Helper T cells
Antibodies (made by plasma/effector cells)
Cytotoxic T cells

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4
Q

What is the predominant feature of impaired immune function?

A

infection

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5
Q

What specifically about infections lead us to believe there is an immune impairment?
3

A

More severe and persist longer
Frequent recurrences
Not responsive to ordinarily effective Rx

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6
Q

What is the nomral amount of respiratory tract infections per year for normal healthy kids?

A

4-8

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7
Q

In primary care what are we mostly prescribing for patients with immunodeficiency disorders?

What dont we give?

A

prohylactic treatment

NO LIVE VIRUSE Vaccines

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8
Q

What are the live virus vaccines?

5

A

rotavirus, varicella, MMR, intranasal influenza, and oral polio

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9
Q

If we have to give a patient with an immunodeficiency a blood transfusion what kind of blood should we give them?3

A

irradiated, leukocyte poor, virus free product

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10
Q

What kind of labs would we test for immunodeficiency disorder?
5

A 
Complete Blood Count (cbc) with peripheral smear 
Lymphocyte immunophenotyping
B and T cell types
Quantitative Immunoglobulins
IgA, IgG, IgM
Antibody Titers
Protein Electrophoresis
Serum Protein (SPEP)
  1. CBC with smear
  2. Lymphocyte typing
  3. Igā€™s
  4. Antibody titer
  5. SPEP (protein)
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11
Q

What is XLA/Brutonā€™s characterized by?

3

A
  1. Humoral immunodeficiency
  2. Severe hypogammaglobulinemia(low antibodies/ all kinds are low)
  3. Increased susceptibility to infection
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12
Q

What demographic is XLA/Brutons found in most often?

A

Mostly young boys

Females are carriers

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13
Q

What cell is virtually missing in XLA/Brutons?

A

B cells

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14
Q

What response is normal in XLA?

A

Normal cell mediated T cell response

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15
Q

Common infections in XLA?

3

A

otitis media, sinusitis, and pneumonia (caused by Strep pneumonia and H. Flu)

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16
Q

When do symptoms start in a child who has XLA?

A

6 months, when the mother stops breast feeding and baby isnt recieving her antibodies anymore

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17
Q

What may XLA babies be missing?

A

Tonsils and cervical lymph nodes

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18
Q 
Warning signs:
How many infections of...
UTIs?
Serious sinus infections?
Pneumonia?
Skin infections?
A
  1. 4
  2. 2
  3. 2
  4. 2
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19
Q

What are the treatment options for XLA?

And in what ways can we accomplish that? 2

A

Replacement of immunoglobulin is the cornerstone of treatment of XLA.

  1. IV immunoglobin
  2. Subcutaneous injected Ig
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20
Q

What is Selective IgA Immunoglobulin Deficiency?

A

Decrease in serum levels of IgA (no other Igs are affected)

no other immunodeficiency disorders either

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21
Q

When can we make the diagnosis for Selective IgA Immunoglobulin Deficiency and why?

A

After 4 because antibody levels in younger children can be depressed normally

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22
Q

Whats the most common immunodeficiency in adults?

A

Selective IgA Immunoglobulin Deficiency

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23
Q

What is the pathology of Selective IgA Immunoglobulin Deficiency?

A

Defect in ability of B cells to terminally differentiate and mature into IgA secreting plasma cells

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24
Q

What infections would you find whit an IgA deficiency?

A

Sinus infections
Pulmonary infections
Intesines
Skin..?

Anywhere there are things being secreted

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25
Q

What is the clinical presentation of Selective IgA Immunoglobulin Deficiency?

4 common infections

A

Not much. Usually found incidentally

Usually an increased incidence of recurrent

  1. sinopulmonary infections
  2. GI tract infections
  3. Conjunctiva
  4. Respiratory tract infections
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26
Q

Besides infections what else increases in risk in Selective IgA Immunoglobulin Deficiency?
3

A
  1. Allergies
  2. Asthma
  3. Autoimmune diseases
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27
Q

Treatment for Selective IgA Immunoglobulin Deficiency?

2

A
  1. Avoid giving meds that lower IgA levels such as penecillamine and valproate
  2. Antibiotics for infections and sometimes prophylactic antibiotics if its a severe case
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28
Q

What should we avoid in patients with Selective IgA Immunoglobulin Deficiency?

A

Should not be transfused
Should not be treated with gamma globulin
ā€”Anaphylactic Hypersensitivity may occur
ā€“body wont recognize the IgA since there is such low levels and will create an antibody for it

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29
Q

What is Common Variable Immunodeficiency?

2

A

(CVID) is impaired B cell differentiation with defective immunoglobulin production

  1. Donā€™t differentiate into plasma/effector cells and helper cells
  2. Thus they dont made antibodies. Effector cells doesnt know its an effector cell
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30
Q

What is CVID defined by?

3

A
  1. Very low serum concentrations of IgG (and usually in combination of IgA and IgM)
  2. Little or no response to immunizations
  3. No other immunodeficiency disorders
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31
Q

CVID is not a single disease, its a collection of hypoimmunoglobulin syndromes resulting in many genetic defects

A

Usually presents between 20-40s

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32
Q

What is the pathology behind CVID?

A

Normal numbers of B lymphocytes but they remain immature. They can still bind to antigens and respond with proliferation but they wont become mature plasma cells or mature memory B cells ever

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33
Q

What is the clinical presentation of CVID?

A

Recurrent infections:
Sinopulmonary such asā€¦
ā€”sinusitis, otitis, bronchitis, pneumonia, TB and fungal infections

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34
Q

What is the main cause of death in CVID?

A

respiratory failure

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35
Q

What other things can CVID lead to besdies infections?

3

A

Autoimmunity
Inflammatory disorders
Malignant disease

(chronic lung disease
gastrointestinal and liver disorders
granulomatous infiltrations of several organs
lymphoid hyperplasia
splenomegaly
malignancy)
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36
Q

Describe the time between onset of symptoms and diagnosis of CVID?

A

CVID affects so many organ systems so its often not clearcut. Usually have been evaluated by a lot of specialists before they are diagnosed. 5-7 years before they know

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37
Q

With what symptoms specifically should we consider CVID?

3

A

adults with:

  1. Chronic pulmonary infections (widening of the bronchi)
  2. Chronic intestinal diseases (infections and malabsorption in the GI tract)
  3. Lymphoid malgnancy
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38
Q

How would we treat CVID?

3

A
  1. IV or Sub-Q Igs (premedicate)
  2. Antibiotics (can use to premedicate or at very early signs of infection)
  3. Wait until they are a little older to immunize them but make sure you do it!
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39
Q

XLA specific deficiency?

2

A

Absence of B cells; very low Ig levels

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40
Q

XLA molecular defect?

A

Mutant tyrosine kinase

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41
Q

XLA clinical features?

A

Pyogenic infections

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42
Q

Selective IgA specific deficiency?

A

Very low IgA levels

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43
Q

Selective IgA molecular defect?

A

Failure of heavy-chain gene switching

44
Q

Selective IgA clinical features?

A

Sinus and lung infections

45
Q

CVID specific deficiency?

A

Very low IgG

And/Or IgA, IgM levels

46
Q

CVID molecular defect?

A

A combination of immunodeficiency issues

47
Q

CVID clinical features?

A

Sinus and lung infections

48
Q

What are the functions of T cells?

4

A
  1. Control viral disease
  2. Control fungal disease
  3. Monitor/control development of dysplastic (abnormal development) cellular changes
  4. Provide helper function to B cells for effective humoral response
49
Q

What is Thymic aplasia (DiGeorgeā€™s syndrome) ?

A

Defective development of the pharyngeal pouch system

- No development of thymus, thyroid and parathyroid

50
Q

What does the pharyngeal pouch system give rise to?

1 main thing to remember involving immune system

A
  1. thymus,
  2. thyroid,
  3. parathyroids,
  4. maxilla, mandible, aortic arch, cardiac outflow tract, and external/middle ear
51
Q

What are most cases of hymic aplasia (DiGeorgeā€™s syndrome) caused by?

A

Heterzygous chromosomal deletion at 22

52
Q

What does the absense of the development of thymus, thyroid and parathyroid cause?

A

tetany

Can also cause palate defects

53
Q

What kind of infections will we see with thymic aplasia?

A

Severe viral, fungal, and protozoal infections

54
Q

When does thymic aplasia arise?

A

early in life/infants

55
Q

Two common infections in tymic aplasia?

A

Pneumonia by P. jirovecii

Thrush by C. albicans

56
Q

What is the clinical presentation at birth of thymic aplasia?
3

A
  1. Cardiac abnormalites &
  2. hypocalcemia w/ possible tetany
  3. Facial abnormalities
57
Q

If patients with thymic aplasia survive how would you describe their immune system?

A

Can have a normal humoral immune system but profound T cell absense and reduced CD4 T cells

58
Q

What other symptoms can present with thymic aplasia?

4

A

Esophogeal dysmotility
GU tract anomalies
GI anomalies
Autoimmune disease

59
Q

How do we manage thymic aplasia?

4

A
  1. Immediately treat cardiac abnormalities if there
  2. Control hypercalcemia(other metabolic abnormalities)
  3. Give only irradiated blood (helps prevent rejection/death)
  4. Can do a thymus transplant
60
Q

What is Severe combined immunodeficiency (SCID)?

A

SCID is a group of inherited diseases caused by mutations in different genes whose products are crucial for the development and function of both T and B cells

61
Q

When are combined immunodeficiencies considered severe?

A

When they lead to death in the first year due to overwhelming infection

62
Q

What is the pathology behind SCID?

3

A
  1. Absense of thymic tissue and other lymph tissue (no lymphocytes)
  2. Ig levels are very low
  3. Tonsils and lymph nodes are absent
63
Q

Describe B and T cell function in SCID?

2

A
  1. Both are defective and sometimes completely absent in children

OR

  1. The number of cells are normal but they dont function properly
64
Q

What are the two main types of SCID and What is the most common?

A

X-linked
-constitutes about 75% of cases
Autosomal
-25% of cases

65
Q

What are the characteristics of X linked SCID?

2

A
  1. Normal B cells and greatly reduced T cells

2. Low levels of IgG and IgA, can make IgM (still usually fatal)

66
Q

Characteristics of Autosomal SCID?

A

could be multiple mutations that affect B and T cells

67
Q

Clinical presentation of SCID?

A

Failure to thrive (die within 1-2 years, usually pneumonia)

68
Q

What is the best way to manage SCID?

2

A
  1. Isolation is really the only way

2. Live attenuated viral vaccines CANNOT be given

69
Q

Possible treatments for SCID?

3

A
  1. Bone marrow or cord transplant could restore immunity (have to do in three first months of life to have high success)
  2. IV Ig
  3. Enzyme replacement therapy
70
Q

What are Wiskott-Aldrich and

Ataxia-Telangiectasia?

A

immunodeficiency diseases

71
Q

Specific deficiency of Severe combined immunodef-iciency (SCID) ?

A

Deficiency of both

B-cell and T-cell function

72
Q

Molecular defect of SCID?

4

A
  1. Either defective IL-2 receptor,
  2. defective recombinases,
  3. defective kinases,
  4. absence of MHC proteins
73
Q

What is the specific deficiency of Wiskott-Aldrich?

A

IgM and cellular immunity impaired X-linked disease and thus occurs only in males

74
Q

What is the molecular defect in Wiskott-Aldrich?

A

X-linked disease and thus occurs only in males

75
Q

What is the clinical features of Wiskott-Aldrich?

A

Recurrent pyogenic infections, eczema, and bleeding

76
Q

What is the specific deficiency in Ataxia-telangiectasia?

A

Lymphopenia and IgA deficiency

77
Q

What is teh molecular defect in Ataxia-telangiectasia?

A

Autosomal recessive disease

78
Q

What is the clinical features of Ataxia-telangiectasia?

A

Recurrent infections appear by 2 years of age

79
Q

What is hereditary angioedema?

A

A complement-associated immunodeficiency disease that is autosomal dominant and lacks C1 inhibitor. Without this inhibitor, capillary permeability occurs and edema.

80
Q

Where is the swelling most often seen in hereditary angioedema?
4

A 
Happens in episodes of the:
hands
feet
face 
AIRWAY PASSAGES!
81
Q

What is the clincal presentation of hereditary angioedema?

3

A
  • Episodes of swelling lasting 24-72 hours
  • non-itching
  • increase risk of other autoimmune disease
82
Q

How would we manage hereditary angioedema?

4

A
  1. IMMEDIATE ASSESSMENT OF THE AIRWAY
  2. First line therapy: human plasma-derived C1 inhibitor.
  3. Icatibant
  4. Ecallantide
83
Q

Molecular defect of hereditary angioedema?

A

Too much Cā€™3a, Cv4a, and Cā€™5a generated

84
Q

Secondary immunodecificiencies?

7

A 
Malnutrition
Infection
Cancer
Renal disease
Sarcoidosis
Transplantation recipients
Diabetes Mellitus
85
Q

What is the most common cause of immunodeficiency world wide?

A

Malnutrition

86
Q

What does Protein Energy Malnutrition (PEM) associate with to cause immunodeficiencies?
5

A
  1. Impairment of cell-mediated immunity
  2. Phagocyte function
  3. Complement system
  4. Secretory immunoglobulin A antibody concentrations
  5. Cytokine production
87
Q

What does vitamin A deficiency causes in the immune system?

3

A
  1. Deficiency interferes with epithelial cells function
  2. Ability of certain immune cells to kill organisms and
  3. production of B-cells and T-cells are dependent on VA
88
Q

What does vitamin C deficiency cause in the immune system?

3

A
  1. Decrease in collagen synthesis
  2. Phagocyte oxidative burst activity impaired
  3. Inability of B-cells and T-cells to work properly
89
Q

What does vtamin B12 deficiency cause in the immune system?

2

A

Lowers T-cell counts and lower natural killer cell activity

90
Q

What does vitamine B6 deficiency cause in the immune system?

2

A

Decreased T-cell responsiveness and natural killer cells ability to kill organisms

91
Q

How does malnutrition affect the number of circulating T cells and NK cells?

A

declines T cells

INcreases NK cells

92
Q

How are serum immunoglobin levels and specific antibody response affected in malnutrition?

A

Serum immunoglobulin is normal or increased; however, specific antibody responses are impaired.

93
Q

How are lymphoid organs affected in malnutrition?

A

Primary and secondary lymphoid organs are depleted of cells,

94
Q

Bacteria strategies against acquired immunity?

4

A
  1. Molecular mimicry
  2. Suppression of antibodies
  3. Hiding inside cells- dont present to the antigen. hide wtihin the body until they have produced a higher number to fight
  4. Releasing antigen into bloodstream- renders antibody unable to attack the cell
95
Q

Bacteria strategies against phagocytes?

4

A
  1. Inhibit chemotaxis
  2. Inhibiting phagocytosis- produce toxins that inhibit it- staff
  3. Killing the phagocyte
  4. Colonising the phagocyte
96
Q

What does HIV attack?3

A

CD4 lymphocytes, macrophages, dendritic cells

97
Q

What does measles do and what is the result?

A

Attacks T cells and dendritic cells directly

-Causes low T cell count and low antibody production

98
Q

Immunological abnormalitites that are associated with cancer (either from the cancer itself or from the chemo) 3

A

Chronic Lymphocytic Leukemia
Multiple myeloma
solid tumors

99
Q

What does chronic lymphocytic leukemia do?2

A
  1. hypogammaglobulinemia 60%

2. decrease complement activity

100
Q

What happens in multiple myeloma?

3

A

CD4 cells decrease
Deficits in complement function
deficits in neutrophil function

101
Q

What happens when solid tumors affect the immune funtion?

3

A
  1. decrease function of normal lymphocytes
  2. increase suppressor lymph activity
  3. decrease T cell lymphocytes
102
Q

Why would renal disease affect immune function?

A

decrease in protein = decrease in immunoglobulins

103
Q

How does dialysis affect the immune system?

3

A

reduced t cell fucntion
reduced neutrophil function
dimished antobody production

104
Q

renal patients are often on glucocorticoids. how do these affect the immune system?
2

A

supress phagocytes

decrease circulating T cells

105
Q

What is sarcoidosis characterized by?
3

How does it affect immune cells?4

A

by accumulation of T lymphocytes, mononuclear phagocytes and pulmonary granulomas

  1. decreased CD4+ T cells
  2. Hypergammaglobulinemia - B cell hyperactivity
  3. Decreased CD4+/CD8+ T cell ratio
  4. Sarcoid granulomas made up primarily of CD4+ T cells

Immunology (8 decks)

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