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Clinical Pathology > Immunodeficency > Flashcards

Immunodeficency Flashcards

1
Q

what are the principle function of the immune system

A 
protect from infection.
multilayer defence system
network of pathogen recognition to detect which antigens are self.
effective intracellular communication
multiple mechanism to remove pathogen
self regulation.
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2
Q

Is the innate immune system long term or short term

A

short term

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3
Q

Is the adaptive immune system long term or short term

A

long term

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4
Q

is the innate immune system specific or non specific

A

non-specific

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5
Q

Is the adaptive immune system specific or non specific

A

specific.

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6
Q

What are the 3 components of the innate immune system

A

PPR, Cellular, barrier and chemical mechanisms

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7
Q

What are the 2 components of the adaptive immune system

A

cellular and humoral.

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8
Q

Where are receptors in the innate system found.

A

On cells e.g. macrophages and dendritic cells.

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9
Q

What are the major components of the innate immune system and what do they tell us.

A

detect the type of pathogen e.g. gram +ve or -ve, virus but not the specific pathogen
PRR-pattern recognition receptor.
antimicrobial peptides, cells, complement components, cytokines.

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10
Q

Pattern recognition receptors include

A

TOL like receptors, NOD like receptors, Rigl like receptors, C type lectins, scavenger receptors.

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11
Q

Antimicrobial peptides include

A

defensins, cathelin, protegrin, granulysisn, histatin, secretory leukoprotease inhibitors and probiotics.

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12
Q

Cells

A

macrophages, dendritic cells, Nk cells, NK T cells, neutrophils, eosinophils, mast cells, basophils and epithelial cells.

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13
Q

Complement component

A

classic and alternative complememnt pathways and proteins that bind complement components

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14
Q

cytokines

A

Autocrine, paracrine, endocrine, cytokines and mediate host defines and inflammation as well as recruit, direct and regulate adaptive immune responses

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15
Q

Function of humoural aspect of adaptive immune system

A

– Kill, neutralise and recognise pathogen.

– Deal with extracellular orgnaism

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16
Q

What are the different types of T lymphocytes

A

Killer or cytotoxic T lymphocytes are able to kill.

Helper T lymphocytes secrete growth factors (cytokines) which control immune response: Help B lymphocytes and T lymphocytes (Helper T cells are target of HIV)
Organise other components of the immune system.
Deal with intracellular organism e.g virus.

Suppressor T lymphocytes may damp down immune response

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17
Q

What is the function of antibodies

A 
•	Neutralize bacteria
•	Agglutination 
•	Precipitation of dissolved antigens.
PHAGOCYTOSIS
•	Activate complement
CELL LYSIS
18
Q

What is the function of T cells.

A
  • Cytotoxic t cells bind infected cell
  • Perforin makes holes in cell membrane
  • Infected cell is destroyed.
19
Q

Do B cells deal with intra or extracellular infection

A

extracellular

20
Q

Do T cells deal with intra or extracellular infection

A

intracellular.

21
Q

How does the innate immune system communicate with the adaptive

A

cytokines
cells in the innate system which have engulfed the pathogen will express cytokines which trigger the adaptive immune system- instruct CD4 to make T cells.

22
Q

define immunodeficiency.

A

• Clinical situations where the immune system is not effective enough to protect the body against infection

23
Q

Causes of secondary or acquired immunodeficiency.

A 
stress
surgery/burns
malnutrition
Cancer
Immunosuppressive effect of drug- chemotherapy
irradiation
AIDS.
24
Q

Causes of primary immunodeficiency.

A

very rare- genetical

25
Q

What is IRAK deficiency

A

innate system
IRAK is a toll like receptor recognises bacterial cell wall or viral component.
IRAK through intracellular signalling produces NF-Kb (transcriptional factor)- needed to make cytokines

26
Q

What test determines the function of neutrophils

A

Nitroblue tetrazolium test.
Incubate neutrophil with dye and it should take up the dye creating free oxygen radicals forming dark blue circles.
This does not occur if defective.

27
Q

What type of genetic disorder is the defect in NADPH oxidase complex

A

X-linked- defect in how phagocytes work.

28
Q

How does NADPH oxidase complex work

A

NADPH- oxidase complex trasfers one hydrogen atom across the membrane
This forms free oxygen radicals
causes acidic conditions and a decrease in pH
Acidic pH releases prteolytic enzymes to destroy the infection.

29
Q

Clinical symptoms of chronic granulomatous disease (CGD)

A 
•	Osteomyelitis- bone infection
•	Pneumonia
•	Swollen lymph nodes
•	Ginigivitis- (gum disease)
•	Non-malignant granulomas
•	Inflammatory bowel disease
–	Immune system tries to compensate for decreased neutrophils= secrete chemo/cytokines- so although they are immunodeficient also have overproduction of other mechanisms for coping resulting in IBD.
30
Q

What defects in the complement pathway may result in immunodeficiency

A

lack of a factor C1, C9- without a factor a pore cannot be created in the bacterial membrane.

31
Q

How to diagnose a lack of complement factor deficiency

A
  • Sheep RBC don’t have anything to inhibit complement whereas human RBC do
  • Sheep RBC and patient serum- complement should activate and RBC should lysis.
32
Q

What types of infections of defects in B cells predispose you too.

A

respiratory infections

recurrent bacterial infection with pyogenic organisms.

33
Q

what cell type defect results in X-linked agammaglobulinaemia

A

Primary B cell deficiency

34
Q

Is X-linkes agammagloinaemia a recessive or dominant condition.

A

autosomal recessive.

35
Q

what immunoglobulins are deficient in X-linked agammaglobinaemia

A 
IgA
IgG (subclass deficency)
36
Q

Which cell type deficiency is worse- B or T cell

A

T cell- B cells also require T cell help.

37
Q

What are the common symptoms of T cell deficiency

A

• Symptoms are recurrent infection with opportunistic infections, bacteria, viruses,

38
Q

What is the name of the deficiency which has both B and T cell deficiency.

A

– Severe Combined Immunodeficiency (SCID) syndromes

39
Q

How do you treat SCID (severe combined immunodeficiency)

A

bone marrow transplant.

Gene therapy- replace faulty gene using viral plasmid and patient stem cells, virus incorporates genome into patients.

40
Q

What are the primary T cell deficiencies

A

Severe combine immunodeficency syndromes (SCID)
Adenosine deaminase deficency
Purine nucleoside phosphorylase deficncncy
MHC Class II deficencny
WiskottAldrich Syndrome

Clinical Pathology (45 decks)

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