Immunology 2 Flashcards
What is the mechanism of cyclosporin?
- Calcineurin inhibitor
- Binds CYCLOPHILIN
- Prevents IL2 transcription = Block T cell activation
Recurrent bacterial and enteroviral infections after 6months (decrease maternal IgG)
X-linked agammaglobulinemia (BRUTON)
What is the defect in X-linked agammaglobulinemia
BTK; a tyrosine kinase gene
What is the mechanism of action of Tacrolimus (FK506)?
- Calcineurin inhibitor
- Binds FK506 binding protein FKBP
- Prevents IL2 transcription = Blocks T cell activation
What is the clinical use of Tacrolimus?
- Proriasis
2. Rheumatoid Arthritis
Can live vaccine be given to a patient with X-linked agammaglobulinemia
No its contraindicated
Decrease IgA with normal IgM, IgG levels. Increase susceptibility to giardiasis
Selective IgA deficiency
Asymptomatic patient with Airway and GI infection. ANAPHYLAXIS to IgA-containing product
Selective IgA deficiency
Decrease plasma cells and decrease immunoglobulins caused by defect in B-cell differentiation which usually present after age 2
Common variable immunodeficiency
Increased risk of autoimmune disease, bronchiectasis, lymphoma and sinopulmonary infection caused by a defect in B-cell differentiation
common variable immunodeficiency
What is the cause of Thymic aplasia (DiGeorge Syndrome)
22q11 deletion; failure of 3rd and 4th pharyngeal pouches
When the 3rd and 4th pouches fail to develop what will be absent
Thymus and Parathyroids
Hypocalcemia (tetany), recurrent viral and fungal infection and conotruncal abnormalities (tetralogy of Fallot, Truncus arteriosus)
Thymic Aplasia (DiGeorge syndrome)
What are the findings in Thymic Aplasia (DiGeorge Syndrome)
Decrease T-cells, Decrease PTH, Decrease Calcium and ABSENT Thymic shadow on CXR
Disseminated mycobacterial and fungal infection which may present after BCG vaccine
IL-12 receptor deficiency
What is the cause of IL-12 receptor deficiency and the findings associated
Cause: Decrease Th1 response (autosomal recessive)
Findings: Decrease Interferon-gamma
Cause of Autosomal Dominant Hyper-IgE syndrome Job syndrome) is caused by
Deficiency of Th17 cells due to STAT3 mutation
What is azathioprine?
Antimetabolite precursor of 6-MercaptoPurine
What is the mechanism of azathioprine?
Block nucleotide synthesis ==> Inhibit lymphocyte proliferation
What are the adverse effect of azathioprine?
PANCYTOPENIA
What are the clinical uses of azathioprine?
- Rheumatoid arthritis
- Crohn’s dz
- Glomerulonephritis
- Other autoimmune diseases/conditions
In chronic mucocutaneous candidiasis what is found
- Absent in vitro T cell proliferation in response to
Candida antigen - Absent cutaneous reaction to Candida antigen
What is the cause of Ataxia-telangiectasia
Defect in ATM gene—> failure to repair DNA double strand breaks—-> cell cycle arrest
Triad of Cerebellar defect (Ataxia), spider angiomas (telangiectasia), IgA deficiency
Ataxia-telangiectasia
What infection is associated with Mycophenolate and Mofetil?
Invasive CMV
A patient with Hyper IgM syndrome is at an increased risk of
severe pyogenic infections early in live, pneumocystis, cryptosporidium, CMV
What is defective in Hyper-IgM syndrome (X-linked recessive)
CD40L on Th cells
What are the clinical uses of corticosteroids?
- Autoimmune and Inflammatory disorders
- Adrenal insufficiency
- Asthma
- CLL
- non-Hodgkins lymphoma
What are the adverse effects of corticosteroids?
- Cushing syndrome
- Osteoporosis
- Amenorrhea
- Adrenal cortical atrophy
- Peptic ulcers
- Diabetes
- Psychosis
- Avascular necrosis of the femoral head
- Cataracts
- Hyperglycemia
When CD40L on Th cells is defective what does that lead to
class switching defect
What are the findings in Hyper-IgM syndrome
Normal or Increased IgM
Decrease IgG, IgA, IgE
Failure to make germinal centers
What is the cause of Wiskott-Aldrich syndrome
Mutation in WASp gene
When WASp gene is mutated what happens
Leukocytes and platelets are unable to recognize actin cytoskeleton—-> defective antigen presentation