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37 Cards in this Set
- Front
- Back
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What are the majority of primary immunodeficincies due to? What comes after that?
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B cells - 50%
Combined - 20% Phagocytic 18% Cellular 10% Complement 2% |
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What are the 4 types of Bcell deficiencies?
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1. Bruton's X-linked Agammaglob.
2. Common variable immunodeficiency (CVID) 3. Selective Ig disorders 4. Hyper IgM |
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What is the etiology of Bruton's agammaglobulinemia?
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-Xlinked recessive
-All Ig low or absent. |
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What are symptoms of Bruton's aggamaglob?
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-Acute, recurring pyogenic infections by 6 months old.
-Hi-freq autoimmune connective tissue disorders. |
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What are the lab findings in Bruton's aggamaglob?
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-Bcells very low
-Total Ig low Lymph count normal, but all T |
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How is Bruton's disease treated/
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Antibiotics
Gammaglobulin |
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What is the etiology of CVID?
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-Acquired or inherited
-Bcells recognize Ag, but can't differentiate to plasma cells. |
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What are symptoms of CVID?
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-Chronic, recurrent pyogeni infections at ANY age.
-Hi-freq autoimmune diseases. |
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How does CVID differ from Bruton's?
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Bcells are normal or just slightly decreased.
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What is the most common selective Ig disorder?
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IgA deficiency - shows low sIgA and IgA levels.
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What are the symptoms of sIgA deficiency?
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-Diminished mucosal defense
-Recurring sinopulmonary inf. -Diarrhea -Genitourinary tract infections |
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How is IgA defic. treated?
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Antibiotics
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What is the etiology of HIM (hyper IgM)?
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-Failure of Tcells to make CD40 ligand - all Ig stays M.
-70% X-linked |
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What are symptoms of HIM?
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-Susc. to pyogenic/opportunistic infections.
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What is used to treat HIM?
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Gammaglobulins
Granulocyte-macrophage CSF |
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What are the 2 Tcell deficiencies?
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1. Di George syndrome
2. Nezeloff's syndrome |
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What is the etiology of Di George syndrome?
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-Embryogenesis error
-Abnormal thymus/parathyroid gland/heart. |
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What are symptoms of Di George syndrome?
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-Fungal/viral recurring infectns
-Fish mouth -Weird muscle contractions -Cardiac abnormalities |
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What are the lab findings in Di george?
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-Lymphopenia
-no CMI in tcell function tests -Ig levels are normal -low blood calcim |
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How can Di George syndrome be treated?
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Give thymus transplant from a donor that is younger than 14 weeks to prevent GVHD.
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What is the etiology of Nezelof's syndrome?
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-Genetic error in thymic develp.
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How do symptoms of Nezelof's compare to Di George?
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-Both have recurring viral/fungal infections
-Nezelof is prone to malignancy. |
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What are the 2 types of SCID?
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-X-linked, more common in U.S.
-Autosomal recessive - swiss type |
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What is the problem that makes SCID?
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-Stem cells don't differentiate into T and B cells.
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What are the lab findings in SCID?
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literlaly nothing - aggamaglobulinemia, lymphopenia.
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What are 2 types of partial combined immunodeficiency?
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Scott-aldrich
Ataxia - Telangiectasia |
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What is the etiology of Wiskott aldrich?
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X-linked
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What are the X-linked ones?
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-Bruton's Agammaglobulinemia
-X-linked SCID -Wiskott-aldrich |
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What are the symptoms of Wiskott aldrich?
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-Eczema
-Recurrent infections -Bleeding problems |
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What are the lab findings in Wiskott aldrich?
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-Low Tcells, normal B.
-Low IgM other Ig's might be elevated. -THROMBOCYTOPENIA |
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What is the ataxia telangiectasia a deficiency of?
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Combined T and B cell.
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What unusual parameter is high in ataxia telangiectasia?
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-AFP
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What are appropriate lab tests for B and T cell deficiencies?
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To evaluate immunoglobulins:
-Electrophoresis, IEF -RID -EIA -Rate nephelometry, serology |
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What is CGD?
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chronic granulomatous disease - deficiency of phagocytosis.
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What test is done for abnormalities of phagocytosis?
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NBT test - a dye that indicates action of granules; if they are deficient, it won't change color.
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What type of defiency causes hereditary angioneurotic edema?
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Deficiency of complement regulator - C1 esterase inhibitor.
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What is another complement regulator deficiency?
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PNH - paroxysmal nocturnal hemoglobinuria.
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