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Immunology > Immunodeficiency > Flashcards

Immunodeficiency Flashcards

1
Q

what are 3 outcomes of an infection?

A
  1. resolution
  2. latent infection
  3. chronic infection
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2
Q

what is resolution response to infection?

A
  • Normal Immune response
  • Pathogen cleared
  • Tissue repair
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3
Q

what is latent infection response?

A
  • Normal immune response
  • Pathogen controlled (no replication)
  • Infection can re-occur
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4
Q

what is chronic infection response?

A
  • Defective immune response (aka: immunodeficiency)
  • Pathogen not cleared or controlled
  • ongoing viral replication & infection of new cells
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5
Q

what is the major hallmark for immune deficiency?

A

recurrent infections (especially in young children)

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6
Q

what does SPUR stand for and what is it?

A

= it’s the things that can be signs of immuno deficiency

Serious infections (unresponsive to oral antibiotics)

Persistent infections (early structural damage & chronic infections)

Unusual infections (unusual organisms & sites)

Recurrent infections (2 or 1 major and recurrent minor infections in 1 year)

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7
Q

what are some less major features that may also suggest primary immmune deficiency?

A
  • Weight loss or failure to thrive
  • Severe skin rash (eczema)
  • Chronic diarrhoea
  • Mouth ulceration
  • Unusual autoimmune disease
  • Lymphoproliferative disorders (could be precursor to cancer)
  • Cancer
  • Family history
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8
Q

what is primary immunodeficiency?

A

quite common - immune dysregulation & autoinflammatory disorders & defects in innate & adaptive immmunity

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9
Q

What response causes most of hypersensitivity diseases?

A

humoral response (antibody response)

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10
Q

what are common complications of immunodeficeincy?

A

upper & lower respiratory tract complications

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11
Q

what are polyclonal activators?

A

like super antigens - non specific way to activate T cells

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12
Q

what do pulmonary complications lead to?

A

morbidity & mortality

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13
Q

HIV virus attacks what cell type?

A

immune cells expressing CD4

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14
Q

where does B cell development occur?

A

in bone marrow through series of developmental stages

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15
Q

once fully mature, what do B cells do?

A

they exit the bone marrow & circulate around body via blood stream, lymphatics & secondary lymphoid tissues

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16
Q

what happens if B cell defects?

A
  • Increased susceptibility to extracellular pathogens (bacteria, intestinal parasites), also enteroviruses
  • Delays in growth and development
  • Increased incidence of autoimmune diseases, malignant tumours
17
Q

what is XLA?

A

X-linked agammaglobulinemia
- formed by mutation on BTK gene which blocks differentiation & development of pre-B cells

= this means absence or severe reduction in B lymphocytes and Ig’s of all types

18
Q

how can you diagnose XLA?

A

measure serum Ig levels or number of peripheral B cells in blood
confirmatory test = DNA sequencing of BTK gene locus

19
Q

what is treatment of XLA?

A

Intravenous Immunoglobulin (IVIG) - first case you would prescribe antibiotics for ongoing infections, you can give antibody therapy, if very sick then hemapoetic stem cell therapy to complete replace

20
Q

what is hyper-IgM (HIGM) syndrome?

A

caused by a variety of mutations - commonly in CD40L gene (X-linked recessive)
= it means normal number of B lymphocytes, normal or elevated IgM an decreased IgG
= this means defective interactions between B and Tfh cells so CD4 T cells aren’t stimulated so B cells don’t get swapped to different Ig’s

21
Q

what normally do Tfh hlper cells do?

A

they bind to and activate and increase expression of co-stimulatory molecules that will interact with CD4 T cells

22
Q

what is diagnosis of hyper-IgM syndrome?

A
  • Assess serum Ig levels / the B cell numbers in the blood
  • Assess CD40L expression on activated T cells
  • DNA sequencing of the relevant gene loci (e.g. CD40L, CD40, others)
23
Q

what is treatment of hyper- IgM syndrome?

A
  • Intravenous Immunoglobulin (IVIG)
  • Granulocyte colony stimulating factor (G-CSF) therapy if neutropenia is present
  • Hematopoietic stem cell transplantation (bone marrow or cord blood stem cell) where T cell dysfunction is also present (X-linked HIGM)

*in primary immunodeficiency you wouldn’t want to give them vaccine

24
Q

what are features of T cell immunodeficiency?

A
  • Increased susceptibility to intracellular pathogens (viruses, intracellular bacteria)
  • Delays in growth and development
  • Death at early age if untreated
  • Increased incidence of malignant tumours
25
Q

what is T cell immunodeficiency?

A

developmental problem affecting development of thymus so block in T cell development & differentiation
-> Ig deficiencies often present as well since B/T interactions critical important for normal humoral immunity

26
Q

What is DiGeorge syndrome?

A

when you don’t have functioning T cell as don’t have functioning thymus
- developmental defect whcih causes facial defects & congenital heart defect

27
Q

why does T cell functionality drop with age?

A

thymus shrinks gradually over time

28
Q

what is X-SCID?

A

X-linked severe combined immunodeficiency
caused by mutations on the gene encoding IL gamma chain of several cytokine receptors
->causes infections of all types & unusual skin disease - often early infant death if X-SCID not already identified with wider family tree

29
Q

what is CGD?

A

chronic granulomatous disease - phagocytotic deficiency

  • mutations in gene that code for NADPH oxidase complex components so not effective killing & reduced phagocytotic killing by neutrophils & macrophages
30
Q

what is secondary immune deficiency?

A
  • common
    -often subtle
  • often involves more than 1 component of immune system

like HIV, measles

31
Q

what is HIV?

A
  • significant cause of secondary immune response
  • high mutation rate
  • HIV infects CD4+ cells - CD4 is main target for HIV attachement (which means T cells but also cells like macrophages that present CD4)

CD4 help stabilise reaction between TCR & MHC class 2 with peptide on it - without CD4 then not stable

-over time CD4+ T cells are depleted = progressive immunosuppression

Immunology (6 decks)

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