Immunology Flashcards

1
Q

What are the clinical features of immunodeficiency?

SPUR

A

Serious infections
Persistant infections
Unusual infections
Recurrent infections

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2
Q

What is Kostmann Syndrome?

A

failure of neutrophil maturation so no neutrophils

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3
Q

What is the treatment of Kostmann Syndrome?

A

stem cell transplantation

prophylactic antibiotics and antifungals

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4
Q

What is Leukocyte adhesion deficiency?

A

failure of neutrophil adhesion and trans endothelial migration
i.e. have neutrophils but they cannot bind to the infection

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5
Q

What is Leukocyte adhesion caused by?

A

defect in leukocyte integrins (CD18)

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6
Q

What is Chronic Granulomatous disease?

A

failure to produce free radicals

  • excessive inflammation
  • persistant accumulation of neutrophils, activated macrophages and lymphocytes
  • failure to degrade chemoattractants and antigens
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7
Q

What are phagocyte deficiencies?

A

Chronic granulomatous disease
Leukocyte Adhesion Deficiency
Kostmann Syndrome

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8
Q

How do you diagnose chronic granulomatous disease?

A

nitroblue tetrazolium test

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9
Q

What is the treatment of phagocyte deficiencies?

A
  • aggressive management of infection
  • surgical draining of abscess
  • bone marrow transplant
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10
Q

What is SCID?

A

Severe Combined Immunodeficiency

- unable to produce T cells and B cells so body cannot fight infection

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11
Q

What are the clincial presentations of SCID?

A

present by 3 months of age
failure to thrive
persistant diarrhoea
infections of all types

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12
Q

What causes SCID?

A

Mutation of IL-2 receptor

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13
Q

Why does SCID only present after 3 months?

A

Baby is protected by maternal IgG until then

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14
Q

What is DiGeorge Syndrome?

A

T cell deficiency
- T cells are produce in the thymus however these individuals have an underdeveloped thymus so there is no where for them to mature

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15
Q

What are the consequences of DiGeorge Syndrome?

A
congenital heart defects
cleft palate
failure of thymic development
hypocalcaemia
etc...
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16
Q

What is the treatment for DiGeorge Syndrome?

A

antibiotics

immunoglobin replacement

17
Q

What is a hypersensitivity reaction?

A

immune response that results in bystander damage to the self

18
Q

Breifly outline Type 1-4 hypersensitivity reactions according to the Gel and Coombs Classification?

A

Type 1 - immediate hypersensitivity
Type 2 - direct cell killing
Type 3 - immune complex mediated
Type 4 - delayed type hypersensitivity

19
Q

Desribe Type 1 Hypersensitivtiy?

A

IgE mediated in response to an external antigen
e.g. allergy or asthma
IgE is produced by B lymphocytes with the help of TH2 cells

20
Q

Describe the IgE mediated response to an external antigen?

A
  • IgE - allergen complex binds to mast cells via Fc receptors
  • degranulation of mast cells
  • release of preformed and newly synthesised inflammatory molecules
21
Q

Describe Type 2 Hypersensitivity?

A

Direct killing
activation of complement pathway -> cell lysis -> opsonisation -> antibody mediated phagocytosis
e.g. blood transfusion mismatch

22
Q

Describe the classical, alternative and lectin complement pathway?

A

classical - antigen + antibody + C1 complex
alternative - MBL + mannose pathogen
lectin - spontaneous C3 cleavage

23
Q

What is the treatment for blood transfusion hypersensitivity?

A

plasmapheresis

24
Q

Describe Type 3 Hypersensitivity?

A

Immune Complex
In the presence of excess antigen, antibody antigen complexes get stuck in areas (alveoli and bronchi) and activate complement, attracting macrophages and phagocytes.
e.g. Extrinsic Allergic Alveolitis

25
Q

How is Type 3 Hypersensitivity managed?

A

Avoidance of allergen

Corticosteroids to decrease inflammation

26
Q

Describe Type 4 Hypersensitivity?

A

Delayed
T cell mediated - initial sensitisation to antigen which generates primed effector Th1 cells + memory T cells. Subsequent exposure to antigen = activation of previous T cells and recruitment of macrophages and lymphocytes which release proteolytic enzymes and cause persistent infection
e.g. TB/Sarcoidosis/organ rejection

27
Q

What do Th1 cells cause?

A

normal response to an allergen

28
Q

What do Th2 cells cause?

A

atopic (hay fever, asthma etc..) response to allergen

29
Q

How is the allergic reaction in asthma brought about?

A

Allergen is detected by CD4 cells. This activates T helper cells.
TH2 cells activated in the allergic pathway, TH2 cells activate B cells.
IL4 + IL5 cause B cells to mature to P cells. IgE is released. Mast cells display Fc receptors which Bind IgE.
When IgE binds to Fc receptors there is calcium influx degranulation and release of inflammatory mediators (e.g. histamine) = ALLERGIC RESPONSE.

30
Q

What is measured in acute anaphylactic episodes?

A

Tryptase