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MDSC 321 > Final Immunodeficiencies > Flashcards

Final Immunodeficiencies Flashcards

1
Q

What are the two types of immunodeficiencies?

A

-primary and secondary

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2
Q

What is primary immunodeficiency?

A

-inherited conditions resulting in a defect in leukocyte development or function

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3
Q

What consequences for the patient does primary immunodeficiency cause?

A
  • renders the patient susceptible to cancer and infection

- this susceptibility varies greatly depending on the defect

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4
Q

How many general classes of primary immunodeficiencies are recognized?

A

8 general classes consisting of over 120 specific conditions

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5
Q

List all the deficiencies that are included under primary

A
  • combined B and T-cell
  • mostly B cell
  • mostly T cell
  • myeloid
  • complement
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6
Q

How are primary immunodeficiencies classified?

A

According to the developmental stage of the cells involved

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7
Q

What are the consequences of primary combined B and T cell immunodeficiency? (6)

A
  • SCID
  • severe and recurring infections early in life
  • frequent viral or fungal infections
  • lymphophenia
  • fatal if untreated
  • bone marrow transplant
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8
Q

What is lymphophenia

A

Low circulating lymphocytes

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9
Q

What is ADA deficiency?

A

Mutation in adenosine deaminase

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10
Q

What does ADA deficiency cause?

A

-causes combined B and T-cell immunodeficiency

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11
Q

How does ADA deficiency cause immunodeficiency? (4)

A
  • ADA normally breaks down adenosine
  • if deficient there will be a build up of adenosine and S-adenylhomocyteseine
  • this inhibits ribonucleotide reductase
  • inhibiting DNA replication and high levels of S are cytotoxic
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12
Q

What is autosomal SCID caused by?

A
  • mutation in DNA repair or RAG 1 or 2

- failure to successfully rearrange V(D)J genes

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13
Q

What does autosomal SCID cause?

A

-causes combined B and T-cell immunodeficiency

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14
Q

What causes X-linked SCID?

A

Mutation in IL-R common gamma chain

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15
Q

What does X-linked SCID cause?

A

-combined B and T-cell immunodeficiency

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16
Q

What does the mutation caused by X-linked SCID result in

A

A defect in cytokine signalling

17
Q

What causes X-linked Hyper-IgM Syndrome?

A

Mutation in CD40L

18
Q

What does X-linked Hyper-IgM Syndrome cause?

A

B cell immunodeficiency

19
Q

How does X-linked Hyper-IgM Syndrome cause immunodeficiency? (3)

A
  • low levels of blood IgG, IgA and IgE and elevated IgM
  • lack of CD40L leads to a lack of costimulation of B cells which leads to reduced B cell activation in response to T-dependent antigens
  • failure to produce germinal centres, no class-switching, reduced hypermutation and few memory cells
20
Q

What does Autosomal Hyper-IgM Syndrome cause?

A

B cell immunodeficiency

21
Q

What causes autosomal Hyper-IgM Syndrome

A

Mutation in AID

22
Q

What is the difference between X-linked Hyper-IgM Syndrome and autosomal Hyper-IgM Syndrome?

A

First one primarily affects men and second affects both men and woman

23
Q

What causes Bare-Lymphocyte Syndrome?

A

Lack of MHC2 expression

24
Q

What does Bare-Lymphocyte Syndrome cause?

A

T cell immunodeficiency

25
Q

How does Bare-Lymphocyte Syndrome work? (6)

A
  • APC do not express MHC2
  • peptide is not presented to helper cells
  • failure of positive selection in the thymus-no CD4+ T cells
  • lack of T cell help for B cells
  • generalized immune deficiency and susceptible to infections
  • bone marrow transplant with replaced APC’s
26
Q

What causes TAP-deficiency syndrome?

A

Lack of MHC1 expression

27
Q

What does TAP-deficiency syndrome cause?

A

T cell immunodeficiency

28
Q

What causes DiGeorge Syndrome?

A

No thymus

29
Q

What does DiGeorge Syndrome cause?

A

T cell immunodeficiency

30
Q

What is a phagocyte immunodeficiency? (4)

A
  • chronic granulomatous disease
  • can’t generate ROS thus no hydrogen peroxide
  • can’t kill microorganisms leading to formation of granulomas
  • susceptible to bacterial and fungal infections
31
Q

What tests for chronic granulomatous disease?

A

-NBT and DHR test

32
Q

What is the NBT test?

A
  • nitroblue-tetrazolium test
  • substrate that turns blue in the presence of ROS
  • more blue=more ROS
33
Q

What are secondary immunodeficiencies?

A

-acquired conditions resulting in a defect in leukocyte development or function

34
Q

How do secondary immunodeficiencies come about

A

Various medical treatments or infections, can be transient (drug induced) or permanent

35
Q

What are the mechanisms of HIV? (3)

A
  • occur at mucosal surfaces
  • virions are picked up by DC and transported to the draining lymph node
  • virus transfers to CD4+ helper cells
36
Q

What drug was introduced and helped HIV?

A

AZT

37
Q

What was the second drug introduced for HIV?

A
  • HAART highly active anti-retroviral therapy
  • 2 anti retrovirals + protease inhibitor
  • increases life expectancy

MDSC 321 (21 decks)

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