Term
Light chain genes
Heavy chain genes |
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Definition
Vk/lambda & Jk/lamda
V, D, J |
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Term
| Superantigen definition and example |
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Definition
Bind directly to a V of TCR and MHC Ag
not processed by APC
Staph TSST-1 |
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Term
HLA class II, cellular expression
?cells |
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Definition
| B cells, monocytes, activated T cells, vascular endothelial cells |
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Term
Complement activation
Mannose Lectin binding a/w ? infections |
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Definition
| Salmonella, Listeria, Neisseria, Candida, Crypto |
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Term
2-6 mo y/o, low/absent IgG
Reccurent URInfections & Giardia Infestation |
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Definition
Infantile (Bruton's) X-linked Agammaglobulinemia
CD19 B cells--> absent in peripheral blood,
most common -->Xq22 defect
autos recessive--> defect in u heavy chain, CD79A, CD179B |
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Term
5-6 mo y/o
IgG low, reccurent upper respiratory infections
DDx? Test? |
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Definition
Infantile (Bruton's) --> PB: absent CD19
Transient hypogammaglobulinemia of Infancy - CD19 +
--> ~ up to 2 yrs of age |
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Term
Most common 10 Immunedeficiency
most people--> no symptoms |
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Definition
Selective IgA deficiency, ~ a/w IgG2
Risk --> allergic non hemolytic transfusion reactions |
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Term
Reccurent infections w
1. encapsulated organisms
2. Toxin producing organisms
Test for ddx? |
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Definition
1. IG2/4 subclass deficiency
2. IG1/3 subclass deficiency
Monitoring Ig response to tetanus protein / pneumococcus polysaccharide vaccine |
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Term
< 1y/o , IgM = nml- elevated
IgA/E/G = low |
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Definition
X-linked Hyper-IgM deficiency
defect Xq26--> CD40 ligand (CD154) --> class switch |
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Term
Hypoparathyroidism and congenital heart dz
CD3 + T cells - PB: low / absent |
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Definition
DiGeorge Syndrome
22q11.2 & 10p13 |
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Term
| Adenosine deaminase deficiency |
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Definition
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Term
| Lack of either class I (rare) or class II antigens on the lymphocyte surface |
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Definition
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Term
| < 1y/o, Eczema, thrombocytopenia, reccurent pyogenic infections, low IgM, elevated IgE & IgA |
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Definition
Wiscott Aldrich Syndrome
Xp11.22 |
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Term
2-6 y/o, CD4 cells: reduced # & function
IgA = absent |
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Definition
Ataxia Teleangiectasia --> chrom 11
common --> lymphoreticular malignancies |
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Term
| Reccurent soft tissue infections; impaired wound healing |
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Definition
| LAD I --> CD18 defect--> neutro's transmigration |
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Term
| < 2y/o, Lymphadenitis, hepatosplenomegaly, pneumonia (catalase + bacteria, Aspergillus, Candida) |
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Definition
CGD
Nitroblue Tetrazolium Reduction test, Flowcytometry |
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Term
Hereditary angioedema
Cause?Test? |
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Definition
C1 esterase inhibitor deficiency
Test: Functional C1 esterase inhibitor |
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Term
Transplant Immunosupressants
- OCT3
- Simulect
-Rituximab
-Infliximab, Etanercept |
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Definition
- Anti - CD3
- Anti CD25
- Anti - CD 20
- Anti TNF |
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Term
"paraneoplastic syndromes"
--> common Auto-Ab's |
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Definition
Anti-neuronal nuclear -1 (Hu)
Purkinje cell cytoplasmic Ab (Yo)
neuro syndromes a/w ovarian ca |
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Term
HLA disease associations
B27 |
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Definition
| Ankylosing spondylitis and Reiter's |
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Term
| Klebsiella pneumoniae superantigen |
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Definition
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Term
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Definition
LE cell
neutro-->engulfed Ab coated cell Nc |
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Term
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Definition
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Term
RF ?type of Ab
more sensitive test for RA |
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Definition
IgM--> Anti-IgG (Fc region), mono-/poly-clonal
Cyclic citrullinated peptide |
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Term
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Definition
| Mixed connective tissue dz, 80% F w Raynaud's phenomena & swollen digits |
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Term
1.Nucleolar ANA
2.Centromere pattern |
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Definition
Scleroderma--> anti RNA
CREST variant |
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Term
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Definition
ASCA = Anti-Saccharomyces ceriviseae
~ pANCA |
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Term
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Definition
Wegener's; -->Ab - proteinase 3
PAN / other vasculitides --> Ab - MPO |
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Term
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Definition
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Term
ANCA evaluation - Screen on ETOH fixed neutro's
- cytoplasmic specling =?
- perinuclear/nuclear- =? |
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Definition
- cANCA
- pANCA, ANA, aANCA |
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Term
Specimen run on formalin fixed Neutro's
- Cytoplasmic staining
- Negative
Dx? Cause? |
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Definition
pANCA--> MPO is released into cytoplasm
ANA / xANCA --> run on Hep2 to r/o ANA |
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Term
Hashimoto's
Auto Abs Lympho's , Infiltrate? |
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Definition
Anti-Thyroglobulin Ab (> 8 epitopes) &
Anti-Thyroid Peroxidase(microsomal) = cytotoxic
CD4/8, macs & plasma cells
goiter, a/w HLA-DR5 |
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Term
Grave's dz
Lympho's ? HLA assoc? |
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Definition
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Term
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Definition
Anti-thyroglobulin-50%
Anti-TPO - 90%
LATS--> Long acting thyroid stimulator & Anti-thyroid stimulating hormone |
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Term
Diabetes Type I
Lympho's & Ab's? |
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Definition
CD4/8
Auto-Ab's: Anti-islet cell/insulin/insulin Rc |
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Term
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Definition
PBC (90% F)
also in CAH (10-15%) |
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Term
IgM : polyclonal increase
portal region of liver: Plasma cells
Dx? |
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Definition
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Term
| Anti-Liver-Kidney Microsomes |
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Definition
Autoimmune Hepatitis, Type II
(Cytochrome450D2) |
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Term
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Definition
Anti-Endomysial Ab--> IgA Anti-TTG ELISA
Ag= Tissue transglutaminase |
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Term
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Definition
Anti-Gliadin Ab
Anti-Endomysial Ab
Anti-Reticulin Ab |
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Term
Celiac Dz
a/w? HLA typing? |
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Definition
IgA deficiency => IgA anti-TTG - => IgG Anti-TGG /
IgG Anti Gliadin
HLA DQ2/8 |
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Term
| Circulating Anti-Adrenal Ab's |
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Definition
Autoimmune Adrenal inflammation mononuclear cell infiltrate
Addison's Dz |
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Term
| Anti striated muscle Ab's |
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Definition
Myasthenia gravis
Cross reacts w thymic epithelium
=> 10% a/w Thymoma / thymic hyperplasia |
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Term
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Definition
Myasthenia gravis
--> blocks neuromuscular transmission |
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Term
| Small cell Ca and proximal muscle weakness |
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Definition
Myasthenia gravis, Lambert-Eaton variant
also a/w other autoimmune dz's |
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Term
Subtle onset, loss of motor control
young, increased incidence in cold climates
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Definition
MS, IgG index =
IgGCSF / AlbuminCSF : IgGserum / Albuminserum
myelin basic protein level, oligoclonal banding |
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