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Immunology > Ch 18 Immunodeficiencies > Flashcards

Ch 18 Immunodeficiencies Flashcards

1
Q

What are the five categories that immunodeficiencies are broadly divided into?

A
  • humoral
  • complement
  • Cell-mediated
  • phagocytic
  • combined
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2
Q

reticular dysgenesis

A

defect in mitochondrial adenylate kinase; no development of myeloid or lymphoid lineage

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3
Q

ADA deficiency

A

adenosine deaminase; build up of toxins prevents lymphoid lineage

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4
Q

RAG1/RAG2

A

no V(D)J rearrangement; no pre-BCR/TCR

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5
Q

Common cytokine receptor y-chain or JAK3 deficiency

A

no T or NK cells

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6
Q

CD3 chain deficiency

A

no TCR signaling; no T cells

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7
Q

Bare lymphocyte syndrome

A

defect in MHC I: no pos selection of CD8 cells

defect in MHC II: no pos selection of CD4 cells

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8
Q

DiGeorge syndrome

A

TBX1 defect; no thymus= decreased immunity and no T cells

tx with passive Ig or transplant

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9
Q

Wiskott-Aldrich syndrome

A

defect in WASP; cytoskeleton malformation= no phagocytosis or chemotaxis
tx= abs and antibiotics, HSC transfer

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10
Q

Hyper IgM syndrome

A

no CD40/CD40L–> APCs and TH cells can’t communicate. no Ab to T dpdt Ag, only IgM

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11
Q

Hyper IgE syndrome

A

STAT3 defect impairs IL-6/TGF-B and decreases B cell memory but increases IgE levels

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12
Q

X-linked agammaglobulinemia

A
  • low levels of IgG
  • defect in Btk
  • no development past pre-B stage
  • tx with antibiotics and passive Abs
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13
Q

Selective IgA deficiency

A
  • substitution of IgM for IgA as primary mucosal antibody

- IgA secreting B cells unable to differentiate to the plasma cell stage

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14
Q

Leukocyte adhesion deficiency (LAD)

A
  • abnormality in LFA-1, Mac-1, and gp150/95 integrin adhesion molecules
  • limits leukocyte recruitment
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15
Q

Chronic granulomatous disease

A
  • defect in NADPH oxidative pathway used by phagocytes
  • higher inflammation= granulomas
  • tx IFN-y to induce TNF-a and nitric oxide
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16
Q

APECED

A
  • mutation in AIRE gene
  • escape of autoreactive T cells from thymus causing organ-specific autoimmunity
  • adrenal and thyroid
17
Q

IPEX syndrome

A
  • mutated FoxP3 preventing development of TREG cells
  • autoreactive t cells
  • death before 2 years of age due to sepsis and attacks on key organs
18
Q

Immunodeficiency disorders are treated by replacement therapy:

A
  • replacement of missing protein
  • replacement of missing cell type or lineage
  • replacement of messing defective gene
19
Q

Nude mice

A
  • vestigial thymus (no T cells)

- study effects of transplantation and cancer

20
Q

SCID mouse

A
  • no mature T, B, and NK cells

- generate humanized immune systems following transfer of human HSCs

21
Q

RAG knockout mice

A
  • T and B cells absent

- experimental cancer or infectious disease research

22
Q

Ways to acquire secondary immunodeficiency

A
  1. acquired hypogammaglobulinemia
  2. agent-induced immunodeficiency
  3. extremes of age
  4. malnutrition
23
Q

Makeup of HIV

A

2 RNA genome segments + reverse transcriptase enzyme

24
Q

R5 variants are the ____ form

A

infective

25
Q

X4 is the ___ form

A

progressive

26
Q

Three phases of HIV-1

A
  • acute
  • asymptomatic
  • AIDS
27
Q

Acute

A
  • spike in HIV levels in blood

- brought under control by production of Ab

28
Q

Asymptomatic

A
  • lengthy, years

- gradual decrease in CD4+ T cells and increase in viral load

29
Q

Four specific criteria for AIDS

A
  1. evidence of infection with HIV-1
  2. <200 CD4+ T cells/microL of blood
  3. Impaired/absent DTH reactions
  4. Occurrence of opportunistic infections
30
Q

HIV replication cycle targets

A
  1. Chemokine receptor antagonists
  2. fusion inhibition
  3. integrase inhibition
  4. protease inhibition
  5. RT inhibition
31
Q

HAART

A

combo of drugs with different mechanisms; very effective; only 67% of people had access

32
Q

bnAbs

A

broadly neutralizing Abs; being used to develop vaccine

Immunology (17 decks)

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