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