Term
|
Definition
-lowered IgM
-greater susceptibility to encapsulated bacteria: Streptococcus pneumoniae,
Haemophilus influenzae |
|
|
Term
| Paradoxsmal Nocturnal Hemoglobinuria |
|
Definition
-complement mediated lysis of RBC
-Lack of DAF or MCP or CD59
-blood in urine, esp in morning |
|
|
Term
| Lack of Factor D or properdin |
|
Definition
suceptibility to Neisseria
gonorrhoeae and Neisseria meningitidis |
|
|
Term
| Defect in MAC formation (C5-C9) |
|
Definition
suceptibility to Neisseria
gonorrhoeae and Neisseria meningitidis |
|
|
Term
| Hereditary Angioneurotic Edema |
|
Definition
Lack of C1 INT
-uncontrolled activation of C1 |
|
|
Term
| Chronic Granulomatous Disease |
|
Definition
-nonfunctioning NADPH oxidase
-neutrophils cant produce H2O2
-formation of granulomas
-cant eradicate bacteria and fungus Aspergillus |
|
|
Term
| Leukocyte Adhesion Deficiency |
|
Definition
-genetic defect in CD18
-no phagocyte migration into tissue
-normal B and T cell response
-recurring pyogenic infections
-problems with wound healing
-neutrophilia |
|
|
Term
|
Definition
-defect in vesicle formation and trafficking (CHS1/LYDR)
-defect in NK cells, CD8+, endocytosis, etc
-recurrent bacterial infections
-partial albinism, platelet dysfunction, neurological defects |
|
|
Term
| Bruton's Agammaglobulinema |
|
Definition
-defect in BTK
-x linked
-T cells but no B cells (stops at pre-b cell) |
|
|
Term
|
Definition
-failure of embyronic thymus to develop
-no T cells
-but will have B cells
-will have opportunistic infections |
|
|
Term
|
Definition
-Males
-will only make IgM and IgD
-no germinal centers |
|
|
Term
|
Definition
-Males and Females
-will only make IgM and IgD(?) |
|
|
Term
| autoimmune polyendocrine syndrome |
|
Definition
-lack of AIRE
-autoimmune reactions, esp to endocrine organs |
|
|
Term
| IPEX (immune dysregulation polyendocrineopathy enteropathy |
|
Definition
-lack of FoxP3 (needed for Treg)
-fatal autoimmunity |
|
|
Term
| Hereditary angioneurotic Edema |
|
Definition
-C1 inh deficiency
-spontaneous complement activation leads to subepithelial edema |
|
|
Term
|
Definition
-loss of common g chain on IL receptors (loss of IL-2, IL-4, IL-7, IL-9, IL-15, IL-21)
-no NK cells (IL-21)
-B cells present (but can't class switch via IL-4) |
|
|
Term
|
Definition
-no signaling tyrosine kinase for T cells
-NK cells present
-B cells present (but can't class switch using T cell help) |
|
|
Term
|
Definition
-no MHC class II
-no CD4 T cells
-CD8 cells fine
-B cells present (but can't class switch using T cell help) |
|
|
Term
| Adenosine Deaminase deficiency (ADA) |
|
Definition
-build up of dATP toxic
-no T cells (most effected)
-no B or NK cells |
|
|
Term
| Purine Nucleoside phyophorylase (PNP) |
|
Definition
-build up of dGTP toxic
-no T cells (most effected)
-no B or NK cells |
|
|
Term
|
Definition
| -ppl with abnormally high IgE; more likely to have allergies (type I hypersensitivity rxn) |
|
|
Term
|
Definition
| -caused by type I HS rxn to ag in blood |
|
|
Term
|
Definition
-systemic anaphalxis
-caused by mast cell activation of something other than IgE |
|
|
Term
| Penicillin/Quinidine/Methyldopa rxn |
|
Definition
|
|
Term
|
Definition
-type III HS rxn
-localized under skin
-caused by injection of ag |
|
|
Term
|
Definition
-type III HS rxn
-occurs after too much ab is given to patient |
|
|
Term
| Hypersensitivity pneumonitis |
|
Definition
-type III HS rxn
-ag-ab complex in lungs |
|
|
Term
| Contact dermatistis (posion ivy) |
|
Definition
-type IV HS rxn
-activates Th1 cells (extracellular) and CTl (intracelluar) |
|
|
Term
|
Definition
|
|
Term
|
Definition
-type IV HS rxn
-skin blisters |
|
|
Term
|
Definition
|
|
Term
|
Definition
-causes -type IV HS rxn
-high in asians (HLB 1502) |
|
|
Term
|
Definition
|
|
Term
|
Definition
-type IV HS rxn
(caucasion - HLB 5701) |
|
|
Term
|
Definition
-loss of common g chain on IL receptors (loss of IL-2, IL-4, IL-7, IL-9, IL-15, IL-21)
-no NK cells (IL-21)
-B cells present (but can't class switch via IL-4) |
|
|
Term
|
Definition
-no signaling tyrosine kinase for T cells
-NK cells present
-B cells present (but can't class switch using T cell help) |
|
|
Term
|
Definition
-no MHC class II
-no CD4 T cells
-CD8 cells fine
-B cells present (but can't class switch using T cell help) |
|
|
Term
| autoimmune hemolytic anemia |
|
Definition
-autoimmune disease
IgG and IgM ab bind to complements on RBC |
|
|
Term
|
Definition
-autoimmune disease
-ab develop against extracellular matrix
-most in kidney and lungs |
|
|
Term
|
Definition
--autoimmune disease
-overproduction of thyroid hormone
-ab continously activates thryoid receptor
-bias towards TH2 response |
|
|
Term
|
Definition
-autoimmune disease
-loss of thyroid hormone
-ab stimulate Th1 cells.
-overproduction of ectopic lymphoid tissue causes death of thyroid tissue |
|
|
Term
|
Definition
-autoimmune disease
-death of pancreatic B cells |
|
|
Term
| Systematic Lupus Erythematosus |
|
Definition
-autoimmune disease
-rhumatic disease
-autoab esp to dsDNA
-IgG and complement activation
-most common among asian/african women |
|
|
Term
|
Definition
-autoimmune disease
-rhumatic disease
-chronic episodic inflmmation of joints
-B cell makes rheumatoid factor (Igx) ab for human IgG
-leukocyte infiltration of joint synovium
-treat with anti-TNFa or anti-CD20 |
|
|
Term
|
Definition
--autoimmune disease against myelin sheath
-plasma cell secretes IgG into CSF
-motor weakness, lack of coordination |
|
|
Term
|
Definition
-autoimmune disease
-ab binds AcH receptor on muscle
-lessens neural stimualtion
-progressive muscle weakening |
|
|
