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Year 1 > Immunology - immunodeficiency > Flashcards

Immunology - immunodeficiency Flashcards

1
Q

What are the hallmarks of immune deficiency?

A

Serious Infection: unresponsive to oral antibiotics
Persistent Infection
Unusual Infection
Recurrent Infection

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2
Q

What is secondary immunodeficiency?

A
  • acquired
  • common, subtle
  • inolves more than one component of immune system
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3
Q

What is primary immunodeficiency?

A

rare

congenital

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4
Q

Where can secondary immunodeficiencies arise from?

A

Infection (HIV, measles)
Treatment interventions (cancer therapy, steroids)
Malignancy (cancer of immune system, mets)
Biochem and nutritional disease (malnutrition, diabetes)

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5
Q

What is reticular dysgenesis?

A

Failure of stem cells to differentiate along myeloid lineage
Primary

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6
Q

What is Kotsmann syndrome?

A

Failure of neutrophil maturation

Primary

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7
Q

What is the management of Kotsmann syndrome?

A

Supportive treatment inc. prophylactic antifungals & antibiotics
Mortality 70% w/out definitve treatment
(stem cell transplant or Granylocyte colony stimulating factor. GF stimulat maturation neutrophils)

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8
Q

What is leukocyte adhesion deficiency?

A

Failure to recognise selectins on endothelium surface
Problem with leukocyte integrins
No adhesion or migration

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9
Q

What is the clinical picture of LAD?

A 
Marked leukocytosis (increase WBCs in plasma)
Localised bacterial infections difficult to detect
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10
Q

TRUE/FALSE

Defect in opsonin receptors may cause significant disease

A

FALSE
Opsonin receptors enhance phagocytosis, defect may cause defective phagocytosis but significant redundancy of opsonin receptors means this generally does not cause significant disease

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11
Q

TRUE/FALSE

Any defect of complement or antibody production will also result in decreased efficiency of opsonisation

A

TRUE

antibodies and complement (C3B) are opsonins

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12
Q

What is chronic granulomatous disease?

A

Failure of oxidative killing mechanism.
Impaired killing of intra-cellular micro-organisms

This leads to failure to DEGRADE chemoattractants and antigens. Persistant accumularion of WBCs and lymphocytes

= THIS LEADS TO GRANULOMA FORMATION

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13
Q

What are some features of chronic granulomatous disease?

A 
Recurrent bacterial and fungal infections
failure to thrive 
lymphadenopathy (swelling lymph nodes)
hepatosplenomegaly
granuloma
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14
Q

What is the lab investigation of chronic granulomatous disease?

A

Nitroblue Tetrazolium test

sensitive to products of hydrogen perioxide (product of resp burst)

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15
Q

How does body defend against intracellular organisms?

A

Infected macrophages produce IL-12
IL-12 stimulates TH1,TC&NK cells to release secrete interferon gamma
interferon gamma stimulates TNF which activates NADPH oxidase and respiratory burst

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16
Q

What are the gene defects associated with susceptibility to intracellular bacteria? MACROPHAGE CYTOKINES

A

IL-12 deficit

IL-12 receptor deficit

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17
Q

Tests for phagocyte recruitment?

A

Full blood count
Presence of pus
Expression of neutrophil adhesion molecules

18
Q

Test results for Congenital neutropaenia?

A

Neutrophil count: absent
Pus: absent
Leukocyte adhesion markers: normal
NBT: no phagocytosis

19
Q

Test results for leukocyte adhesion defect?

A

Neutrophil count: increased during infection
Pus: no
LD markers: absent
NBT: normal

20
Q

Test results for chronic granulomatous disease?

A

Neutrophil count: normal
Pus: yes
LD markers: normal
NBT: abnormal

21
Q

What is SCID?

A

Severe combined immunodeficiency
Failure of differentiated stem cells to mature into lymphocytes.
Lots of pathways for this to happen.

22
Q

What is the commonest cause of SCID?

A

X-Linked, mutation of IL 2 receptor

23
Q

What does IL 2 do?

A

Activated T cells and B cells

Activates NK cells

24
Q

What does IL 12 do?

A

Enhances cytotoxic activity - stimulates TH1 cells to release secrete interferon gamma
interferon gamma stimulates TNF which activates NADPH oxidase and respiratory burst

25
Q

What are the clinical features of DiGeorge syndrome?

A

Failure development thymus (pharyngeal pouch embryo)

Hypoparathyroidism

26
Q

What would laboratory investigations of Digeorge syndrome show?

A
  • Absent or decreased no. T cells
  • Normal or increased B cells
  • Normal NK cell numbers
27
Q

What are some disorders of T cell effector (helper and cytotoxic) functions?

A
  • cytokine production
  • cytotoxicity
  • T-B cell communication
28
Q

What are the gene defects associated with susceptibility to intracellular bacteria? T CELL CYTOKINES

A

IFNy (stimulates TNFa) receptor deficit

IFNy deficit

29
Q

What is the function of the IL12/IFNy axis?

A

Defence against intracellular bacteria
Macrophage release IL 12
IL 12 ctivates T helper cells which enhances cytotoxic activity of CD8 and NK cells
IL 12 stimulates production of IFNy (from TH1, cytotoxic T cells and NK) IL IL 12 stimulates production of TNFa (from macrophages)

30
Q

Where can phagocyte deficiencies manifest?

A 
Stem cell prod/differentiation
Neutrophil adhesion/endothelial migration
Recognition pathogens
Phagocytosis and killing
Activation of other components
31
Q

What are the first line investigations of T cell deficiencies?

A
  • Total WBC count
  • Serum immunoglobins (functional effector cells promote creation of these)
  • Quantify lymphocyte populations
32
Q

What does CATCH 22 represent?

A 
Problems associated with DiGeorge syndrome
Cardiac problems
Abnormal face
Thymic dysfunction
Cleft deformities
Hypocalcaemia, hypoparathyroidism
22 mutations chromosome 22
33
Q

What are the SECOND line investigations of T cell deficiencies?

A

Test for
FUNCTION
PROLIFERATION
HIV

34
Q

How do antibody deficiencies present?

A

Recurrent bacterial infections
with COMMON bacteria
Viral infections may occur

35
Q

What is Bruton’s X-linked hypogammaglobulinaemia?

A

Failure B cells to mature

  • NO circulating B cells or plasma cells
  • NO circulating antibodies 6 months after BIRTH!
36
Q

What is selective IgA deficiency?

A

No production IgA (breast milk, tears, mucosal sites, mucus)
1/3rd RECURRENT RESP TRACT INFECTION
2/3 ASYMPTOMATIC

37
Q

What is common variable immune deficiency?

A 
Low IgG, IgA and IgE
Consider immunoglobin function
IgG: agglutination, opsonisation, complement activation
IgE: allergies
IgA: Mucosal sites, breast milk, tears
associated autoimmune disease
38
Q

What are the clinical features of CVID?

A 
Recurrent bacterial infections
Often with severe end-organ damage
Bronchiectasis, persistent sinusitis, recurrent gastrointestinal infection
Autoimmune disease
Granulomatous disease
39
Q

How can B cell deficit be managed?

A
  • Treat infection aggressively
  • Immunoglobin replacement therapy
  • Stem cell transplant sometimes
40
Q

What are the first line investigations of B cells?

A

Total WBC count
Serum immunoglobins
Serum and urine protein ELECTROPHORESIS (measure of immunoglobins/ proteins)

41
Q

What are the second line investigations of B cells?

A

Quantify no. T cells and B cells
Meausre SPECIFIC ANTIBODY FUNCTION to known pathogens
- IgG against tetanus
-Immunise with vaccine and measure antibody
failure to moutn response= defect?

Year 1 (15 decks)

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