Immunodeficency Flashcards

1
Q

What kind of infections do you see in patients with an immunodeficiency of Pattern recognition receptors (PRRs)?

A

Pneumococcus - pneumonia

HSV

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2
Q

What kind of infections do you see in patients with a defective phagocyte NADPH oxidase?

A

Chronic granulomatous disease (CGD)

  • pneumonia
  • abscesses of the skin, tissues, and organs
  • osteomyelitis

NADPH oxidase is critical for phagocyte killing of bacteria through reactive oxygen species.

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3
Q

What kind of infections do you see in patients with Hypocomplementemia?

A

Meningococcus - meningitis

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4
Q

What kind of infections do you see in patients with X-linked agammaglobulinaemia?

A

Recurrent
Sino-pulmonary infections

due to B cell deficiency?

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5
Q

What kind of infections do you see in patients with IFN-γ/IL-12 pathway deficiencies

A

Mycobacterium - Tuberculosis, Salmonella

Deficiency of cytokine production prevents a co-ordinated response between the T cells and innate immune system.

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6
Q

What kind of infections do you see in patients with defects in T cells?

A

Severe Combined Immunodeficiency (SCID) syndromes
- Opportunistic infections

Loss of T cells prevents an appropriate B cell response

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7
Q

Why are defects in B cells usually diagnosed at around 1-2yrs and how are they treated?

A

Maternal IgG protects the baby until then

Usually leads to recurrent bacterial infection with pyogenic organisms.

Treat with antibiotics then IV IgG for life.

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8
Q
Adenosine Deaminase Deficiency
Purine Nucleoside Phosphorylase Deficiency
MHC Class II Deficiency
Wiskott-Aldrich Syndrome
are all examples of what?
A

Primary T cell deficiencies

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9
Q

What does the transfer of specific, high-titre antibody from donor to recipient provide?

A

Passive immunity

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10
Q

What is the purpose of G-CSF/GM-CSF in immune stimulation?

A

Granulocyte-colony stimulating factor (G-CSF)

- Act on bone marrow to increase production of mature neutrophils

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11
Q

What is α-interferon mainly used to teat?

A

Hepatitis C

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12
Q

How do corticosteroids suppress the immune system?

A

Decreased neutrophil margination
Reduced production of inflammatory cytokines
Inhibition phospholipase A2 (reduced arachidonic acid metabolites production)
Lymphopenia
Decreased T cells proliferation
Reduced immunoglobulins production

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13
Q

what are the side effects of corticosteroids?

A

Carbohydrate and lipid metabolism - Diabetes, Hyperlipidaemia
Reduced protein synthesis - Poor wound healing
Osteoporosis
Glaucoma and cataracts
Psychiatric complications

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14
Q

What is Azathioprine (AZA) and what is it used for?

A

Immunosuppressant - Affects T and B cells by impairing DNA synthesis

  • Guanine anti-metabolite
  • also converted into 6-mercaptopurine which halts DNA synthesis

Used to prevent allograft rejection and treat autoimmune disease eg. SLE

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15
Q

What are Cyclosporin A (CyA) and Tacrolimus (FK506)?

A

Calcineurin inhibitors

CyA- Binds to intracellular protein cyclophilin

Tacrolimus (FK506)- Binds to intracellular protein FKBP-12

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16
Q

What is Sirolimus?

A

Sirolimus (rapamycin - macrolide antibiotic)

Binds to FKBP12 which inhibits mTOR

Inhibits T cell response to IL-2

17
Q

What are Basiliximab

and Daclizumab ?

A

IL-2 receptor mABs

- Prevents T cell proliferation

18
Q

What are Calcineurin inhibitors?

A

Calcineurin activates Nuclear factor of activated T-cells (NFAT) which stimulates cytokine gene transcription

Inhibition prevents IL-2 production

= Reversible inhibition of T-cell activation, proliferation and clonal expansion

19
Q

What is Mycophenolate mofetil (MMF) and what is it used for?

A

Immunosuppressant - Affects T and B cells by impairing DNA synthesis

Non-competitive inhibitor of inosine monophosphate dehydroginase
(IMPDH)
- Prevents production of guanosine triphosphate

Used to prevent allograft rejection and treat autoimmune disease eg. SLE

20
Q

What is Methotrexate (MTX) and what is it used to treat?

A

Folate antagonist
Purine synthesis inhibitor

Used to treat Rheumatoid arthritis

21
Q

What is Cyclophosphamide and what is it used to treat?

A

Immunosuppressant

  • forms DNA crosslinks both between and within DNA strands
  • induces apoptosis

used to treat Vasculitis and SLE

22
Q

what are DMARD’s?

A

Disease-modifying antirheumatic drugs

23
Q

What is Tocilizumab and what is it used for?

A

mAB which blocks IL-6 receptor

Used in therapy of RA

24
Q

What is Rituximab and what is it used for?

A

Chimeric mAb against CD20- B cell surface

Used for:
Lymphomas, Leukaemia’s
Transplant rejection
Autoimmune disorders

25
Q

What is adoptive immunotherapy?

A

Bone marrow transplant (BMT)

Stem cell transplant (SCT)

26
Q

What is Omalizumab and what is it used for?

A

mAb against IgE

Used in Rx of asthma

27
Q

What is Mepolizumab?

A

mAb against IL-5

Prevents eosinophil recruitment and activation