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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
What infection is associated with Mycophenolate and Mofetil?
Invasive CMV
26
A patient with Hyper IgM syndrome is at an increased risk of
severe pyogenic infections early in live, pneumocystis, cryptosporidium, CMV
27
What is defective in Hyper-IgM syndrome (X-linked recessive)
CD40L on Th cells
28
What are the clinical uses of corticosteroids?
1. Autoimmune and Inflammatory disorders 2. Adrenal insufficiency 3. Asthma 4. CLL 5. non-Hodgkins lymphoma
29
What are the adverse effects of corticosteroids?
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
30
When CD40L on Th cells is defective what does that lead to
class switching defect
31
What are the findings in Hyper-IgM syndrome
Normal or Increased IgM Decrease IgG, IgA, IgE Failure to make germinal centers
32
What is the cause of Wiskott-Aldrich syndrome
Mutation in WASp gene
33
When WASp gene is mutated what happens
Leukocytes and platelets are unable to recognize actin cytoskeleton----> defective antigen presentation
34
What is the clinical use of (IL2) Aldesleukin?
1. Renal Cell Carcinoma | 2. Metastatic melanoma
35
What is the clinical use of Epoetin alfa (erythropoietin)?
Anemias (especially in renal failure)
36
What is the clinical use of FILGRASTIN (G-CSF)?
Stimulate GRANULOCYTES to recover none marrow and WBC count
37
What is the clinical use of SARGROMASTIN (GM-CSF)?
Stimulate GRANULOCYTES and MONOCYTES to recover none marrow and WBC count
38
What is the clinical use of IFN-alpha?
1. Chronic Hepaptitis B and C 2. Kaposi Sarcoma 3. Malignant Melanoma 4. Hairy Cell Leukemia 5. Condyloma Acuminata 6. Renal Cell Carcinoma
39
What is the clinical use of IFN-beta?
Multiple Sclerosis
40
what is the clinical use of IFN-gamma
Chronic Granulomatous dz
41
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
Chronic granulomatous disease
42
Pt is taking a calcineurin inhibitor and presents to the ED with gingival hyperplasia. Which Rx is the pt taking?
Cyclosporin
43
Which calcineurin inhibitor should not be prescribed if pt is borderline diabetic?
Tacrolimus
44
What is another name for Sirolimus?
Rapamycin
45
What is the mechanism of action for Sirolimus?
MTOR inhibitor Binds FKBP Prevents IL2 response = Block T cell activation and B cell differentiation
46
What is the clinical uses of Sirolimus?
Prophylaxis for Kidney transplant
47
What are the adverse effects of Sirolimus
1. Pancytopenia 2. Insulin resistance 3. Hyperlipidemia 4. NOT NEPHROTOXIC
48
Which 2 drugs block IL2?
Basiliximab and Daclizumab
49
What is the mechanism for Basiliximab?
1. Monoclonal Ab | 2. Blocks IL2R
50
What is the clinical use of Basiliximab?
Prophylaxis for kidney transplant rejection
51
What are the adverse effects of Basiliximab?
1. Edema 2. HTN 3. Tremor
52
Chediak-Higashi syndrome is caused by
Defect in lysosomal trafficking regulator gene (LYST)
53
The defect in Lysosomal trafficking regulator gene will lead to
Microtubule dysfunction in phagosome-lysosome fusion (autosomal recessive)
54
Recurrent infection by staphylococci and streptococci, partial albinism, peripheral neuropathy, progressive neurodegeneration, infiltrative lymphohistiocytosis
Chediak-Higashi syndrome
55
Lab findings of Giant granules in granulocytes and platelets. Pancytopenia and Mild coagulation defect is found in
Chediak-Higashi syndrome
56
What enzyme degrades 6-MP?
Xanthine oxidase
57
What is the MOA for Mycophenolate and Mofetil?
1. REVERSIBLY inhibit IMP dehydrogenase ==> | Prevent purine synthesis of B and T cells
58
What are the clinical uses for Mycophenolate and Mofetil?
1. GI upset 2. Pancytopenia 3. HTN 4. Hyperglycemia 5. < Neurotoxic and nephrotoxic
59
Chronic granulomatous disease is caused by
Defect in NADPH oxidase that causes decrease reactive oxygen species and decrease respiratory burst in neutrophils (X-linked recessive most common)
60
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
Chronic granulomatous disease
61
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
Chronic granulomatous disease
62
What are some examples of corticosteroids?
``` Beta-methasone Methyl-prednisone Prednisone Dexamethasone Fluticasone Hydrocortisone Cortisone ```
63
What is the mechanism of action of corticosteroids?
1. Inhibit NF-kappaB 2. Suppress B and T cell function (dec. transcription of many cytokines) 3. Induce T cell apoptosis
64
What are the clinical uses of corticosteroids?
1. Adrenal insufficiency 2. Asthma 3. CLL 4. non-Hodgkins lymphoma
65
What is the reason behind artificial leukocytosis?
Demargination of WBC
66
What may develop in pts if corticosteriods are w/d abruptly after chronic use?
Adrenal insufficiency
67
6-MP inhibits, which enzyme?
PRPP aminotransferase
68
What is the clinical use of 1. Romiplostin (thrombopoietin analog) 2. Eltrombopag (thrombopoietin receptor agonist)
Thrombocytopenia
69
What is the clinical use of Oprelvekin (IL-II)
Thrombocytopenia
70
What cancer drug treats CLL and MS
Alemtuzumab
71
What does alemtuzumab target?
CD52
72
Bevacizumab is used to treat......
1. Colorectal cancer 2. Renal Cell Carcinoma 3. non-Small Cell Lung Cancer
73
VEGF is the target of which cancer drug?
Bevacizumab
74
What Rx is used to treat: 1. Stage IV colorectal cancer 2. Head and Neck Cancer
Cetuximab
75
EGFR is the target marker for which drug?
Cetuximab
76
The treatment for B cell non-Hodgkins lymphoma, CLL, Rheumatoid arthritis, ITP, Pemphigus bugari is....
Rituximab
77
CD20 is the target of which drug?
Rituximab
78
What is the clinical use of Trastuzumab?
Breast cancer, Gastric Cancer
79
What is the target of Trastuzumab?
HER2/neu
80
What drugs can be used to treat IBD, Rheumatoid arthritis, Ankylosing spondylitis, Psoriasis?
Adalimumab Certolizumab Golimumab Infliximab
81
``` What is the target of the following drugs: Adalimumab Certolizumab Golimumab Infliximab ```
Soluble TNF-alpha
82
Relapsing MS is treated with.....
Daclizumab (or Basiliximab)
83
CD25 (part of IL-2 receptor) is the target for which drug?
Daclizumab

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