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Immunology 2 > Immunodeficiency > Flashcards

Immunodeficiency Flashcards

1
Q

difference between primary and secondary immunodeficiencies

A

primary- congenital- rare

secondary- acquired- common

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2
Q

what are the 4 components of the immune response that can be targeted in immunodeficiency

A
  1. t cells
  2. b cells
  3. phagocytes
  4. complement
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3
Q

what types of infections are typical for each component of the immune system?

A

t-cell deficiencies lead to intracellular infections of mycobacteria, viruses, and fungi

all other deficiencies lead to extracellular bacterial infections

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4
Q

name some warning signs of primary immunodeficiencies

A
  1. recurrent ear/sinus infections, pneumonia, or other infections w/in a year
  2. poor response to antibiotics
  3. recurrent deep skin lesions
  4. failure to thrive
  5. family history
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5
Q

describe methods to detect B-cell deficiencies

A
  1. serum immunoelectrophoresis
  2. quantification of serum Igs
  3. quantification of B cells via flow cytommetry
  4. dectection of specific Abs (Schick test)
  5. induction of in-vivo b-lymphocyte differentiation
  6. lymph node biopsy
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6
Q

describe methods to detect t-cell deficiencies

A
  1. DTH to common atigens
  2. total lymphocyte count
  3. quantification and classification using Ab/FACS
  4. proliferation in response to alloantigens- mixed lymphocyte reaction
  5. in vitro analysis of cytokine production
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7
Q

describe evaluation of phagocyte function

A
  1. total granulocyte/monocyte count
  2. chemotaxis assay
  3. assay for phagocytosis of opsinized particles
  4. test for superoxide generation using NBT
  5. assay for cytokines/response to cytokines
  6. antigen processing/ presentation
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8
Q

describe evaluation of complement system

A
  1. total hemolytic complement assay- CH50
  2. immunoassay for individual components
  3. neutrophil chemotaxis w/ complement subunits
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9
Q

how might B cells develop deficiencies if nothing is wrong with them?

A

if helper t cells are damaged and cannot facilitate class switching, b cell repsonses will be impaired

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10
Q

brutons disease

A

b cells fail to develop congenitally

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11
Q

XLA

A

x linked disease where b cells fail to develop d/t a defective gene encoding btk, which is an important signaler in the progression of b cell precursors

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12
Q

hyper-Igm syndrome

A

increased IgM but defective coding for either CD40 or CD40L means no helper t cell interaction for class switching

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13
Q

common variable IgG/IgA immunodeficiencies

A

low IgG or IgA w/ impaired Ab production in response to infection/vaccine

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14
Q

what is the most common primary immunodeficiency?

A

IgA- leads to increased upper respiratory infections

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15
Q

why are deficiencies where only cell mediated immunity is affected rare?

A

b/c t cells are so important in activating b cells, if you impair t cells you impair b cells

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16
Q

digeorge syndrome

A

d/t to an absent or very small thymus, w/ severity depending on the amount of thymus present

17
Q

bare lymphocyte syndrome

A

defects in genes needed for MHC class 2 lead to a lack of CD4 cells and severe SCID

18
Q

TAP1 or TAP2

A

defects in these genes lead to a lack of MHC class 1 and no CD8 cells

19
Q

wiskott-aldrich syndrome

A

mutations in WAS protein result in an impaired T cell activation

20
Q

ataxia telangiectasia

A

caused by mutations that damage ATM protein important for DNA repair, causing impaired lymphocyte development

21
Q

define SCID and some its most common causes

A

severe combined immunodeficiency- affects both T and B cells

  1. stem cell defects in the y chain common to many cytokine receptors
  2. ADA enzyme leading to toxicity of purine metabolite buildup which inhibit DNA synthesis
  3. defects in enzymes involved in recombination, thus cannot produce functional Igs or TCRs
22
Q

what are the 2 types of phagocyte deficiencies?

A

intrinsic- related to the inherent properties of the phagocyte, such as differentiation, chemotaxis, or intracellular killing

extrinsic- deficiency in Ab or C, immunosuppression

23
Q

cyclic neutropenia

A

pts have a low neutrophil count fo 3-6 days in a 21 day cycle

24
Q

leukocyte adhesion deficiency

A

leukocytes have difficulty adhering to endothelium at sites of inflammation

25
Q

chediak-higashi syndrome

A

phagosomes do not fuse with lysosomes

26
Q

chronic granulomatous disease

A

defect in genes for NADPH oxidase, causing defective intracellular killing

27
Q

interferon gamma responsiveness examples

A

inferferon gamma receptor deficiency

jobs syndrome- failure of Th cells to produce IFN-y

28
Q

defects in C3 are prone to what infections

A

encapsulated- pneumococcus, streptococcus, neisseria

29
Q

defects in the MAC complex are prone to what infections

A

mengiococcus (neisseria)

30
Q

hereditary angioedema

A

continuous complement activation d/t C1q inhibitor deficiency

31
Q

paroxysmal nocturnal haemoglobulinuria

A

decay accelerating factor deficiency causes lysis of RBC precurors

32
Q

defects in C1, 2, 4 lead to what?

A

immune complex diseases b/c the Ag/Ab complexes cannot be bound

33
Q

what is the most serious C deficiency?

A

C3 deficiency

34
Q

describe HIV/AIDS infection

A

HIV-1 or HIV-2 viral infection enters the body and has a preference for CD4 cells and monocytes. HIV is a retrovirus and uses reverse transcriptase to encode its own DNA into the host. eventually, these cells will become activated, and generate viral products w/ this DNA. Eventually, CD4 cells are depleted such that opporunistic infections are free to take hold. when this happens, the infection has progressed to clinical AIDS

35
Q

how do B and T cells change as we age?

A

T cells:

thymus transitions to fat and thus new t-cells are no longer created, leaving you only with your existing memory t cells. additionally, t cell expansion reduces with age

B cells

  1. change to autoantigen specificity
  2. less specificty
  3. IgG to IgM

some of these changes are d/t the changes in T cells

36
Q

describe some treatments for immundeficient pts

A
  1. antibiotics
  2. prophylactic antibiotics
  3. IVIG
  4. bone marrow transplant
  5. gene therapy

Immunology 2 (6 decks)

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