Virology A8: Immune Deficiency Diseases

Front 1

What systems can get attacked in immune deficiency diseases?

Back 1

1) B cells, T cells, or both
2) Phagocytic cells
3) Complement

Front 2

1) What is primary immune deficiency?
2) What are the four ways you can get a secondary immune deficiency?

Back 2

1) Inherited defects of immune system
2) Infections by viruses/bacteria, malnutrition, cancer, cytotoxic drugs/chemo

Front 3

What is the biggest cause of immune deficiency worldwide?

Back 3

Malnutrition

Front 4

What are the four main effects of immune deficiency?

Back 4

1) Susceptible to infectious disease
2) Susceptible to opportunistic pathogens
3) Get recurrent infections at a young age
4) Failure to thrive

Front 5

1) What kind of bacteria primarily affect those with antibody deficiency? Why?

2) Why don't you get viral infections if you have an antibody deficiency?

Back 5

Extracellular, because antibodies are the ones that take care of the extracellular diseases (antibodies can't get inside the cell).

2) T cells are still active and getting rid of them

Front 6

If you have antibody deficiency, what kind of infections can you get?

Back 6

Pyogenic bacteria, Giardia

Front 7

If you have T cell deficiency, what kind of diseases can you get?

Back 7

Diseases caused by bacteria, viruses, protozoa, intracellular bacteria

Front 8

If you have combined B cell and T cell defects, what kind of diseases can you get?

Back 8

All - you're screwed

Front 9

If you have phagocytic cell dysfunction, what kind of diseases can you get?

Back 9

Recurrent pyogenic bacterial skin abscesses, periodontitis

Front 10

What are the major pyogenic bacteria?

Back 10

1) Staph aureus
2) Strep pyogenes

Both gram positive cocci

Front 11

Periodontal disease is a hallmark of what type of dysfunction?

Back 11

Neutrophil

Front 12

1) If you have complement deficiency, what kind of infection will you get?

2) What is type 3 hypersensitivity?

Back 12

1) Pyogenic bacteria, immune complex disease (type 3 hypersensitivity reaction)

2) Immune complex diseases - complexes deposited in tissues result in complement activation, PMN activation, platelet aggregation (glomerulitis, vasculitis, arthritis) - serum sickness, Arthus reaction (local antigen excess, IC deposited at site)

Front 13

B cell deficiency disease:

1) When does X-linked agammaglobulinemia occur?
2) What does isolated IgA deficiency occur? Common variable immunodeficiency?

Back 13

1) Before the pre-B cell converts to immature B cell

2) Before immature B cell converts to mature B cell

Front 14

What are the four primary antibody deficiency diseases?

Back 14

1) X-linked hypogammaglobulinemia
2) Common variable immunodeficiency
3) Hyper-IgM syndrome
4) Selective IgA deficiency

Front 15

X-linked hypogammaglobulinemia:

1) What kind of disease is it?
2) What is it also known as?
3) When does it present? Why at this age?
4) Symptoms in bloodwork?
5) What causes it?
6) Who does it occur in?
7) Prognosis?

Back 15

1) Primary antibody deficiency
2) Bruton's
3) ~6 months - maternal antibodies are protective up until that point
4) Low serum Igs, B cells low to absent - true B cell failure, don't make antibodies
5) Mutation on btk gene
6) Boys, because it's X-linked
7) Poor

Front 16

Common variable immunodeficiency:

1) What kind of disease is it?
2) What kind of inheritance?
3) When does it present?
4) Symptoms in bloodwork?
5) What's going on with the B cells?
6) How severe is it compared to Bruton's?
7) What other diseases are you at risk for?
8) Prognosis

Back 16

1) Primary antibody deficiency
2) Variable
3) Early in life
4) Low serum Igs, but not completely absent
5) Normal in number, but abnormal in function
6) Less
7) Autoimmunity, luekemia/lymphoma
8) Decent

Front 17

1) What peak do you NOT see in Bruton's bloodwork?

2) What will you see in a lymph node biopsy?

Back 17

1) No gamma peak
2) Absence of follicles

Front 18

1) How do X-linked hypogammaglobulinemia and Common Variable Immunodeficiency present?

2) What is the treatment?

Back 18

1) Recurrent pyogenic bacterial infections - pneumonia, otitis media, giardiasis

2) Pooled human IgG (gamma globulin), has to be given monthly

Front 19

What does Giardia cause and what kind of disease is it opportunistic for?

Back 19

Opportunistic for primary antibody diseases (Bruton's and Common Variable Immunodeficiency), causes diarrhea, malabsorption, induces failure to thrive.

Front 20

Hyper Ig-M syndrome:

1) What is it linked to?
2) What causes it?
3) What do you see in bloodwork?
4) Status of B cells?
5) What are the side effects?
6) What do you treat with?

Back 20

1) X-linked
2) Lack of CD40 ligand on T cells (PROBLEM IS WITH THE TH2 CELLS)- failure of class switch
3) High serum IgM, low IgG and IgA
4) B cells normal
5) Recurrent bacterial infections
6) Gamma globulin IV

Front 21

Selective IgA deficiency:

1) What makes this disease unique?
2) Symptoms?
3) Status of B cells?
4) What is found in 1/3 patients?
5) Symptoms
6) Treatment?
7) Increase in caries due to IgA deficiency?

Back 21

1) Most common immunodeficiency (1/500)
2) Low to very low IgA
3) B cells normal
4) IgA antibody
5) Most asymptomatic, increased bacterial respiratory infections, eczema rash, allergies, giardisis
6) Antibiotics as needed
7) NO

Front 22

T cell deficiency diseases:

1) At which stage in T cell development do you get severe, combined immunodeficiency?

2) When do you get DiGeorge's syndrome?

3) When do you get AIDS?

Back 22

1) When the T cell progenitor doesn't develop into the pre-T cell

2) There is no thymus development

3) Attack of CD4 T cells

Front 23

DiGeorge's Syndrome:

1) What type of disease is this?
2) What happens in this disease?
3) What causes it? Inherited?
4) Other symptoms?
5) Status of T cells and B cells?

Back 23

1) T cell deficiency
2) Thymus just doesn't develop - defective embryogenesis of 3rd and 4th pharyngeal pouch, deficient development of thymus and parathyroids
3) Not inherited - chromosomal abnormality - deletion affecting **chromosome 22q11**
4) T cell count is low, B cell count is normal but they don't function normally

Front 24

DiGeorge's Syndrome continued:

4) Other symptoms?
5) Status of T cells and B cells?
6) When does it present?
7) What kind of diseases do you get with it?
8) What does a lymph node biopsy look like?

Back 24

4) **facial dysmorphism**, cardiovascular malformations, **neonatal tetany resulting from hypocalcemia secondary to hypoparathyroidism**
8) Normal follicles, but nothing inside - no T cells
5) T cell count VERY low. B cells normal, but deficient function (not activated by T cells).
6) Very early in life
7) Recurrent bacterial infections

Front 25

Severe Combined Immunodeficiency Deficiency (SCID)

1) What kind of genetics results in this?
2) What happens to tissues?
3) T and B cell status?
4) What infections come with it?
5) Treatment?

Back 25

1) 60% - X linked cytokine receptor mutation. **20% - adenosine deaminase deficiency** (buildup of nucleotide metabolism in cell, toxic to lymphocytes)
2) Thymic aplasia, atrophy of lymphoid tissue
3) Both extremely low
4) Recurrent chronic fungal, viral, bacterial infections
5) Bone marrow graft, **gene therapy** (most successfully treated disease)

Front 26

What is neutropenia? What is it due to?

Back 26

Very low numbers of neutrophils in the blood, due to transient or bone marrow failing

Front 27

Leukocyte adhesion deficiency:

1) What kind of disease is this?
2) What happens?
3) Symptoms?

Back 27

1) Neutrophil dysfunction
2) Disease that affects adhesion factors that promote extravasation of neutrophil to get out into bloodstream
3) Periodontitis

Front 28

Lazy leukocyte deficiency:

1) What kind of disease is this?
2) What happens?
3) Symptom?

Back 28

1) Neutrophil dysfunction
2) Neutrophil doesn't want to move to area of infection
3) Periodontitis

Front 29

Chediak-Higashi disease:

1) What kind of disease is this?
2) What happens?
3) Symptom?

Back 29

1) Neutrophil dysfunction
2) Defective lysosome-phagosome fusion (neutrophil usually phagocytosis and then fuses with lysosomes + granules to degrade contents - if lysosomes don't get in, can't kill bacteria)

Front 30

Chronic granulomatous disease:

1) What kind of disease is this?
2) What happens?
3) Symptom?

Back 30

1) Neutrophil dysfunction
2) Defective respiratory burst - defects of NADPH oxidase, the main enzyme
3) Gingivitis, granulomas (non-caseating lung granuloma), recurrent infections with Staph aureus

Front 31

1) What is the hallmark of every neutrophil disease?

2) What are some other diseases?

Back 31

1) Periodontitis.

2) Recurrent pyogenic infections, abscesses

Front 32

Complement deficiency diseases: what happens if you have a deficiency of:

1) C1 inhibitor - what is caused, why, and where does it mainly present?

2) C1, C2, C4

3) C3

4) C5-C9

Back 32

1) Angioedema - complement fires off randomly, results in a lot of extracellular fluid forming. Presents in orofacial area

2) Immune complex disease deposited in skin, joints

3) Recurrent bacterial infections

4) Recurrent Neisserial infections - usually get recurrent meningitis

Front 33

Neisseria:

1) Main virulence factor?
2) Sensitive to?
3) Gram?
4) Anaerobic/aerobic?
5) Cultured on?
6) What does it cause?

Back 33

1) Endotoxin
2) Drying
3) -
4) Aerobic
5) Thayer Martin
6) Gonorrhea (second most commonly caused infectious disease in US), most common cause of acute meningitis in older kids, adolescents, young adults

Front 34

What are the four x-linked immunodeficiency diseases? What part of the chromosome are they found on?

Back 34

1) X-linked chronic granulomatous (neutrophil dysfunction, NADPH oxidase) P part of chromosome


ON Q PART:
2) X-linked SCID - thymic and lymphoid atrophy and aplasia, T and B cells very low, X-linked cytokine receptor mutation and ADA deficiency

3) X-linked agammaglobulinemia - mutation on btk genes

4) X-linked hyper IgM syndrome - due to T cells not having CD40L, failure of class switch

Front 35

What is the most common primary immunodeficiency syndromes? Least common?

Back 35

Most common - selective IgA deficiency

Least common - Bruton's

Front 36

What are the main causes of secondary immune deficiency? (4)

Back 36

1) Viruses - HIV, adenovirus (interferes with MHC-1 expression), measles (causes lymphocytes to arrest in G1)

2) Bacteria

3) Malnutrition (most common cause worldwide)

4) Cancer, chemo, cytotoxic drugs

Front 37

Protein-calorie malnutrition:

1) Main cause of?
2) T cell status?
3) What happens to lymphocytes?
4) Skin test?
5) Humoral immunity?
6) Phoagcytes?

Back 37

1) Secondary bacterial infections
2) Low
3) Reduced responsiveness
4) Anergy
5) Mostly normal
6) Phagocytic dysfunction

Front 38

Sickle cell disease:

1) What kind of immune deficiency, primary or secondary?
2) What are patients susceptible to?
3) What kind of disease is this?

Back 38

1) Secondary
2) Infections with ENCAPSULATED organisms, like Strep pnuemoniae
3) Functional hyposplenism

Front 39

Multiple myeloma:

1) What is it?
2) What happens?
3) How does it look over a radiograph?
4) On a spectrophotometer?

Back 39

1) Plasma cell tumor
2) Monoclonal gammopathy - starts off with one abnormal cell that just reproduces a ton of the same abnormal antibody, all useless! In response, immune system tones down production of normal antibody, get secondary antibody deficiency because of this.
3) Like holes in the skull
4) Huge IgG peak

Front 40

CLL:

1) What is it?
2) What is it associated with?
3) What symptom will you see associated with it?

Back 40

1) Chronic lymphocytic leukemia
2) T cell deficiency aggravated by administration of cytotoxic drugs
3) Candidiasis