Primary immunosuppression Flashcards

1
Q

Where do T-lymphocytes develop?

A

Thymus

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2
Q

Where do B-lymphocytes develop?

A

Bone marrow

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3
Q

What are consequences of immunodeficiency?

A

Susceptiblity to infections
Susceptibility to cancer
Increased incidence of autoimmunity

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4
Q

What causes primary immunodeficiency?

A

Genetic or congenital disorders

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5
Q

What abnormalities can be caused by primary immunodeficiency?

A

Part of immune system is missing or not functioning properly

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6
Q

Where can abnormalities be in primary immunodeficiency?

A

Innate immune system
Stages of lymphocyte development
Responses of mature lymphocytes to antigenic stimulation

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7
Q

What happens as a result of agammaglobulinaemias?

A

Decrease in all serum Ig isotypes

Reduced B cell numbers

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8
Q

What infections are people with B cell deficiency susceptible to vs people with T cell deficiency?

A

B - Pyogenic bacteria, Enteric bacteria and viruses, some parasites
T - Pneumocystis jiroveci, viruses, atypical mycobacteria, fungi

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9
Q

What are causes of primary immunodeficiency?

A

Mutations
Polymorphisms
Polygenic disorders

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10
Q

What is a polymorphism?

A

Change in one of two or more variants of a particular DNA sequence

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11
Q

What is a polygenic disorder?

A

Disorder caused by combined action of more than one gene

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12
Q

What is SCID?

A

Severe Combined ImmunoDeficiency - disorders that affect both T and B cells

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13
Q

Is SCID screened for?

A

Yes

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14
Q

What alleles can cause polymorphic conditions?

A

Human leukocyte antigen

Mannan-binding lectin

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15
Q

What are common polygenic disorders affecting immunity?

A

Common variable immunodeficiency
IgA deficiency
Specific antibody deficiency

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16
Q

How is SCID diagnosed?

A

Clinical presentation - Recurrent infection, unusual infections, diarrhoea, rashes
History - FH of neonatal death or consanguinity
Very low lymphocyte count

17
Q

How is antibody deficiency diagnosed?

A

Presentation - Presents later in life, chronic or recurrent bacterial rresp infection
Antibody levels - Measure IgG, IgM, and IgG, exclude causes of secondary immunodeficiency

18
Q

How is primary immunodeficiency treated?

A

Antibiotic prophylaxis

Immunoglobulin replacement therapy if severe