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RESP > Primary Immunodeficiency 2 > Flashcards

Primary Immunodeficiency 2 Flashcards

1
Q

Outline the life cycle of a T lymphocyte.

A
  1. Produced from stem cells in the bone marrow.
  2. Naive T cells exported to thymus, selection takes place
  3. Mature T cells travel to lymphoid tissue and follicles, where they reside.
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2
Q

Two main classifications of T cells?

A
  1. CD4+

2. CD8+= Cytotoxic T cell

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3
Q

Function of CD4+ cells?

A
  1. Immunoregulatory functions
    - Stimulate CD8 & B cells
    - Produce cytokines
  2. Recognise peptides on MHC class II molecules
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4
Q

Function of CD8+ cells?

A
  1. Recognise peptides on MHC class I molecules
  2. Directly kill pathogens via perforin release
  3. Important in viral and tumour defence
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5
Q

What are the functions of B lymphocytes? (2)

A

Antibody production & presentation

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6
Q

What are the two types that B cells differentiate into?

A
  1. Memory B cells

2. Plasma cells that produce antibodies

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7
Q

Functions of antibodies? (4)

A
  1. Identify pathogens
  2. Stimulate other components of immune system (complement, NK, phagocytes)
  3. Neutralise toxins
  4. vital for Bacterial infection defence
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8
Q

What is reticular dysgenesis?

A

Defect of haemopoetic stem cells which causes lack of production of

  • Neutrophils
  • Macrophages
  • Lymphocytes
  • Platelets

Fatal if not corrected with bone marrow transplant

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9
Q

What happens in severe combined immunodeficiency (SCID)?

A

Failure to produce lymphocytes

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10
Q

Some common clinical presentations of SCID?

A
  1. Recurrent infections
  2. Persistent diarrhoea
  3. Failure to thrive
  4. Unusual skin disease (graft vs host disease)

Dont get acutely unwell until 3 months of age because of maternal IgG in colostrum

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11
Q

What is the commonest form of SCID? (genetic)

A

X linked

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12
Q

What is the result of X linked SCID?

A

IL2 mutation

  • Failure to produce T cells
  • Immature B cells produced
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13
Q

Treatment for SCID?

A
  1. Prophylactic- Antibiotics, antifungals, IV immmunoglobin.

2. Definitive- stem cell transplant

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14
Q

SCID is suitable for gene therapy. T/F?

A

True

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15
Q

What happens in Digeorge syndrome?

A

Failure in thymic development- Developmental defect of 3/4th pharyngeal pouch

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16
Q

What are the clinical features of Digeorge’s? (5)

A
  1. Congenital heart defects
  2. Cleft palate
  3. T lymphopenia
  4. Hypothyroidsim & hypocalcaemia
    5 Psychiatric disorders, developmental delay
17
Q

Digeorge’s is caused due to a deletion at chromosome 22q11. T/F?

A

True

18
Q

Digeore syndrome makes someone susceptible to what?

A

Recurrent infections- bacterial, fungal and viral

19
Q

What do investigations reveal in Digeorge? (immune cell count)

A
  1. Absent or low T cell count
  2. Normal/High B cell- Low IgG, E, A
  3. Normal NK
20
Q

Management Digeorge’s?

A
  1. Correct metabolic/cardiac issue
  2. Prohylactics
  3. Aggressive antibiotics
  4. Ig replacement therapy
21
Q

What are the disorders of T Cell effector functions?

A
  1. Cytokine production
  2. T-B cell communication
  3. Cytotoxicity
22
Q

What are the two main T cell effector disorders?

A
  1. IL12 Deficiency

2. IFNy deficiency

23
Q

Clinical presentations of T cell effector disorders? (3)

A
  1. Recurrent infections- TB, atypical mycobacteria, BCG, aspergillus
  2. Malignancies at young age
  3. Autoimmune diseases
24
Q

What are the investigations for T cell deficiencies?

A
  1. 1st line
    - WBC
    - Serum immunoglobins, protein electrophoresis
    - Quantify sub lymphocyte numbers
  2. 2nd line
    - Test T cell activation & proliferation
    - Additional lymphocyte lineage test
    - HIV test
25
Q

What happens in Brutons X linked hypogammaglobulinaemia?

A

Failure to produce mature B cells

  • No circulating B cells
  • No plasma cells
  • no circulating antibody after 1st 6 months
26
Q

What is Selective IgA deficiency?

A

No IgA produced.
Increases risk of coeliac disease 10x.
Usually asymptomatic

27
Q

What happens in common variable immunodeficiency? (CVID)?

A

Low IgG, IgE, IgA.

28
Q

Clinical features of CVID?

A
  1. Recurrent bacterial infections
  2. Autoimmune disease
  3. Granulomatous disease
29
Q

Investigations for B cell deficiencies?

A

Same as T cell

  • WBC
  • Serum Ig, protein electrophoresis
  • Quantify T cell & B cell levels
  • Specific antibody responses to pathogens
30
Q

Management for B cell deficiencies?

A
  1. Aggressive antibiotics
  2. Antibody replacement therapy
  3. Stem cell transplant

RESP (25 decks)

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