Deficiency in Adaptive Immunity (complete) Flashcards Preview

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Flashcards in Deficiency in Adaptive Immunity (complete) Deck (24)
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1
Q

Describe generally primary immunodeficiencies

A
  • Means a disease probably has a genetic cause
  • Ask yourself: does it exist at birth or not? (Congenital)
  • Ex: if thymus were congenitally dysfunctional
2
Q

Describe generally secondary immunodeficiencies

A
  • Some known process outside the immune system caused the deficiency
  • Think: ACQUIRED
  • Ex: HIV, measles, malaria, EBV
3
Q

What types of infections would you see with pure B cell deficiency?

A
  • High grade EXTRACELLULAR bacterial pathogens (e.g. Staph, H flu, Stept)
  • Just think about the type of immune response that involve B cells
4
Q

What types of infections would you see with pure T cell deficiency?

A
  • Intracellular pathogens (viruses, certain bacteria, yeasts, fungi)
  • Just think about the type of immune response that involve T cells :D then you got it!
5
Q

Describe the clinical features which are part of DiGeorge syndrome, excluding immunological features

A

1) Hypertelorism
2) Down-slanting eyes
3) fishmouth deformity
4) Micrognathia
5) Low-set ears

6
Q

Discuss the incidence of IgA deficiency. What are the associated syndromes?

A

1 in 500 — most common immunodeficiency disease

10x more frequent in people w/ celiac

1) Diarrhea
2) Sinopulmonary infections
3) Increased frequency/severity of allergies

7
Q

Describe the immunological problem of the Nude mouse. What is the human immunodeficiency condition it resembles?

A
  • Have a genetic mutation and have no thymus, can’t make the stroma —-» no T cells
  • Also have no hair
  • Very similar to DiGeorge Syndrome, but it’s a different mutation

These mice reject grafts b/c there are no T cells — all around very useful for research

8
Q

What is the enzyme absent in certain cases of SCID?

A

Adenosine Deaminase

9
Q

Discuss possible approaches to replacing adenosine deaminase

A

1) Enzyme replacement therapy
2) Blood transfusions of irradiated red cells (RBCs have ADA— radiation gets rid of remaining B/T cells)
3) Purified stem cell transplant (otherwise potential for GvH)

10
Q

Discuss transplantation therapy in immunodeficiency diseases. What are some complications?

A

Always potential for GvH disease

  • BM transplant has 50% success rate in SCID — purified stem cells are preferred to avoid GvH
11
Q

What are the principle tests for determining a B cell deficiency?

A

1) Serum protein electrophoresis
2) Quantitative IgG, IgA, IgM levels
3) Specific Abs to prior immunizations
4) ABO isohemagglutinins

12
Q

What are the principle tests for determining a T cell deficiency?

A

1) Skin test w/ recall Ag panel

2) Total lymphocyte count

13
Q

What are the principle tests for determining a phagocyte deficiency?

A

1) WBC count, differential, morphology

2) NBT test – oxidative burst

14
Q

What are the principle tests for determining a complement deficiency?

A

1) CH(50)

2) Assay for C1inh (inhibitor)

15
Q

Where in the lymph node are B cells located?

A

Germinal Centers

16
Q

Where in the lymph node are T cells located?

A

Paracortex

17
Q

Describe the contents of IVIG

A

Aka: Human Immunoglobulin

  • about 99% IgG
  • It’s donated by lots of people
18
Q

Describe the routes of administration for IVIG

A
  • IV use — effective, expensive; often in short supply

- SC use — can be done at home

19
Q

For which conditions would IVIG be useful as replacement therapy?

A

Those w/ B cell deficiencies

Eg:

  • Bruton
  • SCID
  • HyperIgM
  • CVID
20
Q

What are 2 viruses which are immunosuppressive in humans?

A

1) Measles
2) Mono
3) CMV infection
4) AIDS

21
Q

Describe Severe Combined Immunodeficiency Disease

A
  • T and B cell deficient
  • Block in development of lymphoid stem cell
  • X linked: defect in gamma chain —» IL-2 receptor problems
22
Q

Describe X-linked (Bruton)n agammaglobulinemia

A
  • Normal T cells
  • Low to absent B cells
  • Due to developmental block btwn pre-B cells and B cells — most pts have pre-B cells in BM, but deficient in mature B cells
  • IgG has less than 10% of normal, no IgA or IgM
  • This is why OPV isn’t used in the US
23
Q

Describe X-linked hyperIgM syndrome

A
  • It is how it sounds
  • Too much IgM w/ low IgG and IgA
  • Defect in IgM-to-IgG switch mechanism
24
Q

Describe Common Variable Immunodeficiency (CVID)

A
  • Normal #s of pre-B and mature B cells
  • Difficult to trigger to make a specific Ab — IgG is low
  • Can be diagnosed for the first time in pts up to 50yo
  • Main phenotype: recurrent bacterial infections
  • Tx: IVIG, SCIG
  • Causes: unknown