Immunology 2 Flashcards

1
Q

What is the mechanism of cyclosporin?

A
  1. Calcineurin inhibitor
  2. Binds CYCLOPHILIN
  3. Prevents IL2 transcription = Block T cell activation
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2
Q

Recurrent bacterial and enteroviral infections after 6months (decrease maternal IgG)

A

X-linked agammaglobulinemia (BRUTON)

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3
Q

What is the defect in X-linked agammaglobulinemia

A

BTK; a tyrosine kinase gene

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4
Q

What is the mechanism of action of Tacrolimus (FK506)?

A
  1. Calcineurin inhibitor
  2. Binds FK506 binding protein FKBP
  3. Prevents IL2 transcription = Blocks T cell activation
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5
Q

What is the clinical use of Tacrolimus?

A
  1. Proriasis

2. Rheumatoid Arthritis

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6
Q

Can live vaccine be given to a patient with X-linked agammaglobulinemia

A

No its contraindicated

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7
Q

Decrease IgA with normal IgM, IgG levels. Increase susceptibility to giardiasis

A

Selective IgA deficiency

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8
Q

Asymptomatic patient with Airway and GI infection. ANAPHYLAXIS to IgA-containing product

A

Selective IgA deficiency

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9
Q

Decrease plasma cells and decrease immunoglobulins caused by defect in B-cell differentiation which usually present after age 2

A

Common variable immunodeficiency

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10
Q

Increased risk of autoimmune disease, bronchiectasis, lymphoma and sinopulmonary infection caused by a defect in B-cell differentiation

A

common variable immunodeficiency

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11
Q

What is the cause of Thymic aplasia (DiGeorge Syndrome)

A

22q11 deletion; failure of 3rd and 4th pharyngeal pouches

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12
Q

When the 3rd and 4th pouches fail to develop what will be absent

A

Thymus and Parathyroids

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13
Q

Hypocalcemia (tetany), recurrent viral and fungal infection and conotruncal abnormalities (tetralogy of Fallot, Truncus arteriosus)

A

Thymic Aplasia (DiGeorge syndrome)

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14
Q

What are the findings in Thymic Aplasia (DiGeorge Syndrome)

A

Decrease T-cells, Decrease PTH, Decrease Calcium and ABSENT Thymic shadow on CXR

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15
Q

Disseminated mycobacterial and fungal infection which may present after BCG vaccine

A

IL-12 receptor deficiency

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16
Q

What is the cause of IL-12 receptor deficiency and the findings associated

A

Cause: Decrease Th1 response (autosomal recessive)
Findings: Decrease Interferon-gamma

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17
Q

Cause of Autosomal Dominant Hyper-IgE syndrome Job syndrome) is caused by

A

Deficiency of Th17 cells due to STAT3 mutation

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18
Q

What is azathioprine?

A

Antimetabolite precursor of 6-MercaptoPurine

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19
Q

What is the mechanism of azathioprine?

A

Block nucleotide synthesis ==> Inhibit lymphocyte proliferation

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20
Q

What are the adverse effect of azathioprine?

A

PANCYTOPENIA

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21
Q

What are the clinical uses of azathioprine?

A
  1. Rheumatoid arthritis
  2. Crohn’s dz
  3. Glomerulonephritis
  4. Other autoimmune diseases/conditions
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22
Q

In chronic mucocutaneous candidiasis what is found

A
  1. Absent in vitro T cell proliferation in response to
    Candida antigen
  2. Absent cutaneous reaction to Candida antigen
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23
Q

What is the cause of Ataxia-telangiectasia

A

Defect in ATM gene—> failure to repair DNA double strand breaks—-> cell cycle arrest

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24
Q

Triad of Cerebellar defect (Ataxia), spider angiomas (telangiectasia), IgA deficiency

A

Ataxia-telangiectasia

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25
Q

What infection is associated with Mycophenolate and Mofetil?

A

Invasive CMV

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26
Q

A patient with Hyper IgM syndrome is at an increased risk of

A

severe pyogenic infections early in live, pneumocystis, cryptosporidium, CMV

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27
Q

What is defective in Hyper-IgM syndrome (X-linked recessive)

A

CD40L on Th cells

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28
Q

What are the clinical uses of corticosteroids?

A
  1. Autoimmune and Inflammatory disorders
  2. Adrenal insufficiency
  3. Asthma
  4. CLL
  5. non-Hodgkins lymphoma
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29
Q

What are the adverse effects of corticosteroids?

A
  1. Cushing syndrome
  2. Osteoporosis
  3. Amenorrhea
  4. Adrenal cortical atrophy
  5. Peptic ulcers
  6. Diabetes
  7. Psychosis
  8. Avascular necrosis of the femoral head
  9. Cataracts
  10. Hyperglycemia
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30
Q

When CD40L on Th cells is defective what does that lead to

A

class switching defect

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31
Q

What are the findings in Hyper-IgM syndrome

A

Normal or Increased IgM
Decrease IgG, IgA, IgE
Failure to make germinal centers

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32
Q

What is the cause of Wiskott-Aldrich syndrome

A

Mutation in WASp gene

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33
Q

When WASp gene is mutated what happens

A

Leukocytes and platelets are unable to recognize actin cytoskeleton—-> defective antigen presentation

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34
Q

What is the clinical use of (IL2) Aldesleukin?

A
  1. Renal Cell Carcinoma

2. Metastatic melanoma

35
Q

What is the clinical use of Epoetin alfa (erythropoietin)?

A

Anemias (especially in renal failure)

36
Q

What is the clinical use of FILGRASTIN (G-CSF)?

A

Stimulate GRANULOCYTES to recover none marrow and WBC count

37
Q

What is the clinical use of SARGROMASTIN (GM-CSF)?

A

Stimulate GRANULOCYTES and MONOCYTES to recover none marrow and WBC count

38
Q

What is the clinical use of IFN-alpha?

A
  1. Chronic Hepaptitis B and C
  2. Kaposi Sarcoma
  3. Malignant Melanoma
  4. Hairy Cell Leukemia
  5. Condyloma Acuminata
  6. Renal Cell Carcinoma
39
Q

What is the clinical use of IFN-beta?

A

Multiple Sclerosis

40
Q

what is the clinical use of IFN-gamma

A

Chronic Granulomatous dz

41
Q

A patient with an increased susceptibility of catalase positive organism (eg. Staphylococci, Pseudomonas aeruginosa, Aspergillus fumigatus, Candida albicans., Enterobacteriaceae (Klebsiella, Serratia) due to defect in NADPH oxidase has

A

Chronic granulomatous disease

42
Q

Pt is taking a calcineurin inhibitor and presents to the ED with gingival hyperplasia. Which Rx is the pt taking?

A

Cyclosporin

43
Q

Which calcineurin inhibitor should not be prescribed if pt is borderline diabetic?

A

Tacrolimus

44
Q

What is another name for Sirolimus?

A

Rapamycin

45
Q

What is the mechanism of action for Sirolimus?

A

MTOR inhibitor
Binds FKBP
Prevents IL2 response = Block T cell activation and B cell differentiation

46
Q

What is the clinical uses of Sirolimus?

A

Prophylaxis for Kidney transplant

47
Q

What are the adverse effects of Sirolimus

A
  1. Pancytopenia
  2. Insulin resistance
  3. Hyperlipidemia
  4. NOT NEPHROTOXIC
48
Q

Which 2 drugs block IL2?

A

Basiliximab and Daclizumab

49
Q

What is the mechanism for Basiliximab?

A
  1. Monoclonal Ab

2. Blocks IL2R

50
Q

What is the clinical use of Basiliximab?

A

Prophylaxis for kidney transplant rejection

51
Q

What are the adverse effects of Basiliximab?

A
  1. Edema
  2. HTN
  3. Tremor
52
Q

Chediak-Higashi syndrome is caused by

A

Defect in lysosomal trafficking regulator gene (LYST)

53
Q

The defect in Lysosomal trafficking regulator gene will lead to

A

Microtubule dysfunction in phagosome-lysosome fusion (autosomal recessive)

54
Q

Recurrent infection by staphylococci and streptococci, partial albinism, peripheral neuropathy, progressive neurodegeneration, infiltrative lymphohistiocytosis

A

Chediak-Higashi syndrome

55
Q

Lab findings of Giant granules in granulocytes and platelets. Pancytopenia and Mild coagulation defect is found in

A

Chediak-Higashi syndrome

56
Q

What enzyme degrades 6-MP?

A

Xanthine oxidase

57
Q

What is the MOA for Mycophenolate and Mofetil?

A
  1. REVERSIBLY inhibit IMP dehydrogenase ==>

Prevent purine synthesis of B and T cells

58
Q

What are the clinical uses for Mycophenolate and Mofetil?

A
  1. GI upset
  2. Pancytopenia
  3. HTN
  4. Hyperglycemia
  5. < Neurotoxic and nephrotoxic
59
Q

Chronic granulomatous disease is caused by

A

Defect in NADPH oxidase that causes decrease reactive oxygen species and decrease respiratory burst in neutrophils (X-linked recessive most common)

60
Q

A patient with an increased susceptibility of catalase positive organism (eg. Staphylococci, Pseudomonas aeruginosa, Aspergillus fumigatus, Candida albicans., Enterobacteriaceae (Klebsiella, Serrati) due to defect in NADPH oxidase has

A

Chronic granulomatous disease

61
Q

In lab findings where there is abnormal dihydrorhodamine test (decrease green fluorescence) and also Nitroblue tetrazolium dye reduction test fails to turn blue suffers from

A

Chronic granulomatous disease

62
Q

What are some examples of corticosteroids?

A
Beta-methasone
Methyl-prednisone
Prednisone
Dexamethasone
Fluticasone
Hydrocortisone
Cortisone
63
Q

What is the mechanism of action of corticosteroids?

A
  1. Inhibit NF-kappaB
  2. Suppress B and T cell function (dec. transcription of many cytokines)
  3. Induce T cell apoptosis
64
Q

What are the clinical uses of corticosteroids?

A
  1. Adrenal insufficiency
  2. Asthma
  3. CLL
  4. non-Hodgkins lymphoma
65
Q

What is the reason behind artificial leukocytosis?

A

Demargination of WBC

66
Q

What may develop in pts if corticosteriods are w/d abruptly after chronic use?

A

Adrenal insufficiency

67
Q

6-MP inhibits, which enzyme?

A

PRPP aminotransferase

68
Q

What is the clinical use of

  1. Romiplostin (thrombopoietin analog)
  2. Eltrombopag (thrombopoietin receptor agonist)
A

Thrombocytopenia

69
Q

What is the clinical use of Oprelvekin (IL-II)

A

Thrombocytopenia

70
Q

What cancer drug treats CLL and MS

A

Alemtuzumab

71
Q

What does alemtuzumab target?

A

CD52

72
Q

Bevacizumab is used to treat……

A
  1. Colorectal cancer
  2. Renal Cell Carcinoma
  3. non-Small Cell Lung Cancer
73
Q

VEGF is the target of which cancer drug?

A

Bevacizumab

74
Q

What Rx is used to treat:

  1. Stage IV colorectal cancer
  2. Head and Neck Cancer
A

Cetuximab

75
Q

EGFR is the target marker for which drug?

A

Cetuximab

76
Q

The treatment for B cell non-Hodgkins lymphoma, CLL, Rheumatoid arthritis, ITP, Pemphigus bugari is….

A

Rituximab

77
Q

CD20 is the target of which drug?

A

Rituximab

78
Q

What is the clinical use of Trastuzumab?

A

Breast cancer, Gastric Cancer

79
Q

What is the target of Trastuzumab?

A

HER2/neu

80
Q

What drugs can be used to treat IBD, Rheumatoid arthritis, Ankylosing spondylitis, Psoriasis?

A

Adalimumab
Certolizumab
Golimumab
Infliximab

81
Q
What is the target of the following drugs:
Adalimumab
Certolizumab
Golimumab
Infliximab
A

Soluble TNF-alpha

82
Q

Relapsing MS is treated with…..

A

Daclizumab (or Basiliximab)

83
Q

CD25 (part of IL-2 receptor) is the target for which drug?

A

Daclizumab