Small Group Flashcards
RAG deficiency
B and T deficient but normal NK
Adenosine deaminase deficiency
T,B & NK cells absent)
JAK-3 deficiency
T cells
only
These patients have defective terminal B-cell differentiation, absence of plasma cell formation and antibody production. Early Bcell development is quite normal and is reflected by normal (even increased numbers) of Bcells in peripheral blood (detected by phenotyping) but a marked reduction in terminal Bcells,
plasma cell and reduced numbers of germinal centers in lymphoid tissue
common variable immunodeficiency (CVI),
The picture
is one of “frustrated” B-cell responses and the defects usually reside in some sort of
defective cytokine signaling or cross talk between B and Th2 cells, which prevents the Bcells
from differentiating to a later stage. Theoretically, abnormal T regs could be
suppressing B cell terminal differentiation.
Early appearance of ‘T-Cell’ type infectious diseases such as disseminated fungus and protoza and the marked decrease in lymphoid cells in her blood and tissues like tonsils and spleen.
severe combined immunodeficiency (SCID).
This type of presentation suggests an early lineage defect in a stem cell line that leads to
impaired production of lymphocytes but not neutrophils, platelets and erythrocytes (there are very rare defects that do involve all lineages). The defect in lymphocyte development is
usually associated with a growth signal or activating signal. Found commonly in SCID are
fundamental interleukin receptor defects that cause very broad defects in immune responses.
or
adenosine deaminase deficiency-retroviral mediated transfer of the adenosine deaminase
defective gene into the bone marrow stem cells of this type of patient can be curative.
Defect in early B-cell development that prevents differentiation and
proliferation of antibody forming cells.
The defect has been localized to the X chromosome and leads to defective tyrosine kinase signaling and subsequent defective or absence of appropriate display of B cell growth and survival receptors in many cases.
How to make a clinical diagnosis? What tests?
X-linked agammaglobulinemia
Inability to produce specific antibodies to the capsular antigens of pathogenic bacteria leads to suboptimal binding to C3b and Fcγ receptors on neutrophils, which, in turn, subsequently causes inefficient phagocytosis and bacterial killing. Persistent bacterial survival leads to clinical infection.
clinical diagnosis can be made simply by doing a serum protein electrophoresis and
absolute quantitation of the deficiency can be done techniques for each Ig isotype.
How can you detect IC in tissue? Describe the lab test.
The inflammatory reaction could be characterized by the use of fluorescinated or peroxidase
labeled anti IgG and anti C3 reagents that will detect immune complexes in tissue
What cytokines may induce muscle aches and
severe fatigue?
IL1 and IL6
once circulating autoimmune complexes of DNA and anti-
DNA are formed, what happens next?
complement and Fc receptor pathways are activated and serve as
generators of inflammation by a final common pathway (neutrophil recruitment).
Neutrophils & Monocyte phagocytosis via FcR or C3b
(also neutrophil recruited by C3a and C5a)
how indirect immunofluorescence of the area you decide to biopsy could explain the pathogenesis of his disease and how direct immunofluorescence could explain the actual etiologic agent of his disease
- indirect immunofluorescence tells you what response was. (IgG response or complement involved)
- direct tells you for specific pathogen…
What might the failure of a control serum with known anti-glomerular antibodies to stain the glomeruli of some patients indicate?
defect in collagen.
Goodpastures (antibody against type IV collagen)
How are the immunodominant peptides of these allergens are usually preferentially presented?
The immunodominant peptides of these allergens are usually preferentially presented in selected (Class II) D MHC loci by dendritic cells.
A common component of many of the environmental antigens is that they contain…
chitin-polysaccharide not found in mammals.
Late phase allergy reaction dep. totally on T cell activation and presence of which cytokines?
IL3, 4, 5, 13, TNF-α, GM-CSF and IL-10.
What does omalizumab and Ipilimumab treat?
omalizumab- (anti IGE antibodies)
Ipilimumab blocks CTLA-4
What are SCIT and SLIT?
Immunotherapy - given subcutaneous(allergy shots, SCIT) or sublingual(SLIT)
Sublingual Immunotherapy SLIT Grastek - Timothy grass Oralair - Kentucky blue, orchard, perennial rye, sweet vernal and timothy grass Ragwitek - Ragweed
Life threatening infections soon after birth
Wasting, Failure to thrive
Lack of Thymic shadow
What cells missing (specifically)?
Severe Combined Immunodeficiency Disease (SCID)
Lack of CD3+, CD4+, CD8+ and lymphocyte response to antigens
What ligands are might someone w X-linked SCID be unable to respond to?
IL2, IL4, IL7, IL9, IL15
missing common gamma chain so failure to activate transcription of specific genes
Describe the RAST test, what is it used for?
Allergen in solid phase,
IgE (serum)
-patients serum added to a cellulose disc with covalently bound allergen
IgE binds to allergen (IgE present in serum binds to allergen)
After washing, radio-labeled anti-IgE added, radioactivity is countered w a gamma counter
In vitro assays should be considered only diagnostic adjuncts to an appropriate clinical setting.
Skin testing and RAST assays can be positive, but the patient may not have clinical symptoms.
Progesterone also stimulates the surface cells of the uterine
endometrium to display (BLANK) which in turn inhibits complement mediated cell death.
Progesterone also stimulates the surface cells of the uterine
endometrium to display decay accelerating factor (DAF or CD55-
see complement lecture) which in turn inhibits complement
mediated cell death
How can IBD be treated?
B. fragilis or B. subtilits?
IBD can be treated by Bacteroides fragilis which induces an anti-inflammatory response
What bacteria would protect against experimental EAE.
B frag. or B sub.
Intestinal bacteria can protect from experimental autoimmune encephalomyelitis (EAE)?
Explain why/how high fat diet induces changes in the microbiota that can promote obesity:
High fat diet affects innate lymphoid cells to secrete IL-22 which induces expression of anti-microbial peptides which will alter the microbiota.
high fat and carbohydrate diet leads to increase in Firmicutes and decrease in Bacteroidetes
