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21 Cards in this Set
- Front
- Back
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Primary Immunodeficiencies are due to what? How about Secondary?
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Primary- due to intrinsic defects; genetically inherited; from the parents; the minority
Secondary- from extrinsic factors; *worldwide biggest cause of immune deficiency is malnutrition (secondary) **in the US, it's drugs |
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#1 thing to look for when considering immune deficiencies?
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infections; recurrent, persistant, severe, unusual
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Categories of Immunodeficiencies
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1. B-cell defects (most common immune deficiencies)
2. T-cell defects 3. Combined T and B- cell defects 4. Phagocyte Defects 5. Complement Defects |
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Most common immunodeficiency is?
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IgA Deficiency
-Asymptomatic or recurrent bacterial infections -the body is able to compensate for the lack of IgA -may get deficiency in IgG subclasses |
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No B-cells
Affects male children Tx with IVIG |
X-linked Hypogammaglobulinemia
-little Ab of all classes |
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Immunodeficiency with Hyper-IgM comes from where?
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A defect in a helper T-cell where you don't get isotype switching, so you make and secrete IgM, but no other isotypes
-remains intravascular, not out in the tissues, so you're more susceptible to infections |
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Which CD is involved in isotype switching?
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-CD40 on B cell and CD40 ligand on the T-cell (has the defect for hyper-IgM)
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In Common Variable Immunodeficiency, what is lacking?
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CVID
-no plasma cells -occurs in 2nd and 3rd decades -recurrent sinopulmonary infections *total lack of most Ig classes |
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DiGeorge Syndrome
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Primary T-cell deficiency
-thymus and parathyroids fail to develop -variable T-cell deficits Triad of hypocalcemia, low T-cells, congenital heart disease |
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SCID
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Severe Combined Immunodeficiency
-"bubble boy syndrome" -lacking both T and B cells -recurrent serious infections -fatal within 2 years w/o tx |
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Most common form of SCID and the reason why we get it?
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X-linked SCID is the most common form of SCID.
-due to a mutated CYTOKINE RECEPTOR (common gamma gene); IL-2,4,7,9,15 all share this cytokine receptor, which are important for the activation and development of T and B cells. |
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What are other ways to get SCID?
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-defect in the RAG genes (which are important for recombination)
-deficiencies in ADA or PNP enzymes (responsible for metabolizing deoxyinosine and guanine, respectively) for making ATP and GTP |
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Wiskott-Aldrich Syndrome
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-Defects in Ig and T-cells
-X linked inheritance -eczema, thrombocytopenia, recurrent infections *abnormal platelets, T cells, and B cells |
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Extrinsic phagocytic disorders and Intrinsic phagocytic disorders
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Extrinsic are outside the realm of the cell itself (bad opsonization)
Intrinsic defect in intracellular killing machinery (chronic granulomatous disease) |
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Chronic Granulomatous Disease describe and what causes it?!
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deficiency in the intracellular killing mechanism
-onset at 2 yrs - x-linked - DEFECT IN NADPH OXIDASE ENZYME; don't get production of ROS so you can phagocytize, but you can't kill once engulfed. -accumulation of phagocytic cells. |
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What causes Leukocyte Adhesion Deficiency, Type I?
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Beta-2 integrin defect!
intergrins- a CAM that allows neutrophils to get into the tissues -unable to get neutrophils to the tissues to eat up organisms, so they stay in the blood (high count), but little pus formation. |
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Complement System Deficiencies, where can you be lacking?
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Almost all the complement proteins
Classical- C1,C2,C4 MB-lectin Pathway- factors B,D, Properidin *all come together at the C3 Convertase made from C3 protein which carries on the cascade **without a terminal protein, you don't get the MAC |
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Something special about Bacteria Niceria
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causes gonnorea and meningitis, is the only genus susceptible to a lack of membrane attack complex. all other bacteria have ways of getting around being lysed by MAC
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Role of C2,C4, C3
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Removal of immune complexes
-cells in the liver and spleen may have receptors for protein fragments -defect in immune complexes, can get immune complex diseases |
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Why do you get Hereditary Angioedema?
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C1-Inhibitor Deficiency!!!
-most important clinical complement deficiency -C1-INH is a complement control protein, so without it, you have too much complement activation and too much bradykinin, an inflammatory mediator -inappropriate inflammation, possible respiratory occlusion |
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Live vaccines...
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don't give live vaccines to those with immune deficiencies!!
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