Term
- what is the most common of all the primary immunodeficiencies?
- what are the signs & when do they appear? |
|
Definition
- selective IgA deficiency (<5mg/dL)
- ~1:250-1000
- recurrent bacterial & viral infections of the respiratory tract
- children usually begin to develop symptoms around 2
- there is also an increased risk of atopy (b.c. more IgE class switching) |
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Term
| anitbodies that activate complement through classical pathway |
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Definition
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Term
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Definition
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Term
| antibodies that participate in ADCC |
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Definition
IgG
- binding of Fc portion to macros, NK cells or neutrophils cause destruction of cells targeted by the antibody |
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Term
| Antibody that is transported across placenta |
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Definition
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Term
| ABs that are naive B-cell receptor |
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Definition
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Term
| ABs that are memory B-cells |
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Definition
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Term
| what cell type is most numerous in granulomas |
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Definition
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Term
Describe Wiskott-Aldrich syndrome:
- inheritance
- typical presentation
- lab values
- pathogenesis
- treatment? |
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Definition
- X-liked recessive
- triad of: recurrent sinopulmonary infections, eczema, and thrombocytopenia
- Low serum IgM; IgG is nearly normal; IgA & IgE are increased
- Patients have defective response to polysaccharide antigens (e.g. pneumococcal) due to cytoskeleton defect not allowing T cells to bind to B cells
- pts may benefit from bone marrow transplant |
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Term
| low IgM, normal IgG, increased IgA & IgE |
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Definition
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Term
| thrombocytopenia, eczema, and recurrent sinopulmonary infections |
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Definition
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Term
| when does ataxia develop in ataxia telangiectasia? |
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Definition
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Term
| pathogenesis of DiGeorge syndrome? |
|
Definition
failure to form 4th & 5th pharyngeal pouches (athymic & no parathyroid glands)
- hypocalcemic tetany & Cell mediated immunity deficiencies
- PCP pneumonia & oral thrush are common infections |
|
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Term
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Definition
| gamma-delta cells: don't need thymus for maturation |
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Term
| why does your arm hurt after a vaccination? |
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Definition
| local immune complex formation (T3H) (aka arthus reaction) |
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Term
Normal IgM, incrased IgG & IgE, No IgA
which types of pathogens are they most susceptible to? |
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Definition
selective IgA deficiency
- enteric pathogens, especially those that never cross the mucosal barrier (e.g. giardia, cryptoporidium). This is because if an enteric bug that goes systemic (think salmonella typhi), the mucosal surfaces will increase IgG to compensate
- also increased atopy b.c. increased IgE |
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Term
| onset of recurrent bacterial infections & decreased antibodies. associated w/ autimmune conditions (esp Addisons, thyroiditis, RA) |
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Definition
| common variable immunodeficiency; onset usually occurs from 10-20 |
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Term
| common variable immunodeficieny |
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Definition
- recurrent bacterial infections & decreased antibodies starting from 10-20y.o.
- some have intact cell immunity, other have variable to severe T-cell deficiencies
- associated w/ autimmune conditions (esp Addisons, thyroiditis, RA) |
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Term
| hyper IgM syndrome pathogenesis |
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Definition
| absence of CD40L on T-cells |
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Term
| how do you differentiate x-linked hypogammaglobulinemia (Bruton's agammaglobulinemia) from common variable immunodeficiency? |
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Definition
XLH = lower B-cells & Px earlier in life
CVI = normal B-cell levels; presents between 10-20yrs old
both have very low levels of immunoglobulins |
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Term
| X-linked hypogammaglobulinemia (Bruton's agammglobulinemia) etiology |
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Definition
| Mutation in Bruton's tyrosine kinase: can't mature B-cells |
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Term
|
Definition
IFN-gamma, TNF-beta, IL-2
IFN-gamma inhibits Th2 |
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Term
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Definition
IL-4,5,6,10,13; TGF-beta
IL-4 & IL-10 inhibit Th1 |
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Term
|
Definition
|
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Term
| immunodeficiency w/ cytoskeleton defect? |
|
Definition
Wiskott-Aldrich syndrome
(Eczema, thrombocytopenia, sinopulmonary infections) |
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Term
most common cause of SCID
what labs do you see? |
|
Definition
IL-2 receptor defect
decreased everything (T cells, B cells, immunoglobulins) |
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Term
| In this tyrosine kinase deficiency, you are more succeptible to intracellular or extracellular pathogens? |
|
Definition
| extracellular b.c. you can't make antibodies in bruton's because you have no B-cells |
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Term
| Describe leukocyte adhesion deficieny |
|
Definition
- Autosomal recessive
- defective CD18 (LFA-1) --> phagocytes can't diapedese --> recurrent bacterial & fungal infections early in life w/ minimal inflammation despite extreme neutrophilia
- early signs include delayed separation of the umbilicus & omphalitis (infection of the chord)
- CD18 is the common beta chain for the beta-2 integrins which are responsible for the tight adhesions of leukocytes to endothelia |
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Term
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Definition
| Leukocyte adhesion deficiency (ADA LFA-1) |
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Term
|
Definition
| CD18 (leukocyte adhesion deficiency) |
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Term
| reccurent, long lasting infections of skin, respiratory tract, lower intestinal tract, mouth, and genital tract. chronic ulcerations. |
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Definition
| leukocyte adhesion deficiency syndrome (CD18) |
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|
Term
inclusions in nucleated blood cells & albinism
pathogenesis? |
|
Definition
Chediak Higashi syndrome (AR, LYST gene)
(defective intracellular trafficking --> impaired phagolysosome fusion; also defective chemotaxis b.c. of microtuble problems) |
|
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Term
| diagnose w/ flow cytometry dihidrorhodamine test |
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Definition
|
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Term
| disseminated candidiasis in diabetic |
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Definition
| myeloperoxidase deficiency (usually clinically silent) |
|
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Term
|
Definition
| polyribitol phosphate capsule conjugated to surface antigen (kids) or diphteria toxoid (adults) |
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Term
|
Definition
| IL5 & TGF-beta (latter from Treg) |
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Term
| secreted by Th2 & would decrease contact dermatitis from poison ivy |
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Definition
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Term
| produced by macrophages and is most important for stimulating an inflammatory reacion & attraction of neutrophils to site of injury |
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Definition
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Term
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Definition
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Term
| things found in mast cell granules |
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Definition
Histamine, heparin, and eosinophil chemotactic factor-A
Histamine is most important for immediate phase. It works through H1 receptors to increase permiability of venules in acute inflammation |
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Term
| Things that can cause mast cell degranulation (6) |
|
Definition
- extremes of temperature (hot or cold)
- Physical injury
- Type 1 hypersensitivity
- C3a, C5a anaphylatoxins
- substance P
- melitin (bee venom)
- many other things (e.g. IL-8) |
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Term
| major basic protein: where is it and what does it do? |
|
Definition
- in eosinophils
- damages cuticles of most helminths |
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Term
| needed for IgE production |
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Definition
|
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Term
| how does desensitization therapy work? |
|
Definition
| administer small amounts of allergen over a period of time to develop an IgG respons --> IgG binds allergen before IgE can |
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Term
| how does cromolyn sodium work? |
|
Definition
| stabilized mast cell membranes --> decrease degranulation |
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Term
| How does theophylline work? |
|
Definition
inhibits phosphodiesterase --> increase cAMP in mast cells --> decreases degranulation (no effect on allergin binding to IgE)
(increasing cAMP in mast cells in also one of the effects of epinephrine in the Tx of anaphylaxis) |
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Term
| name main symptoms of rheumatic fever |
|
Definition
- Pancarditis
- arthritis
- subcutaneous nodules
- chorea
- erythema marginatum |
|
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Term
| amastigotes inside mononuclear cells (monocytes, macrophages) in circulation, lymph nodes, spleen, and bone marrow |
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Definition
| visceral leishmaniasis (Leishmania donovani) |
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Term
| Promotes macrophage intracellular killing |
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Definition
|
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Term
TNF-alpha
-who makes it?
-what does it do? |
|
Definition
- macrophages & NK cells
- cytotoxic to tumor cells, induces cytokine release, and causes cachexia of chronic inflammation. |
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Term
| what immunodeficiency do you get increased atopy with? |
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Definition
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Term
| 3 immunodeficiency disorders where there is decreased phagocytic efficiency |
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Definition
- Chediak-Higashi
- G6PD def
- CGD |
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Term
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Definition
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Term
| complement component needed to clear immune complexes |
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Definition
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Term
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Definition
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Term
|
Definition
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Term
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Definition
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Term
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Definition
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Term
| depigmentation and anesthetic cutaneous lesions |
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Definition
hansens disease (M.leprae)
likes skin and cutaneous nerves |
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Term
| complement component that acts as an opsonin |
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Definition
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Term
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Definition
|
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Term
| describe Th cell activation |
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Definition
| APC presentation of MHC II molecules loaded w/ foreign peptides followed by stimulation w/ IL-1 & IL-6 & TNF-alpha |
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Term
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Definition
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Term
|
Definition
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Term
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Definition
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Term
|
Definition
| toxoid vaccines for Tetanus & Diphtheria |
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Term
| cytokines induced to be released by TSST (& other superantigens) |
|
Definition
IL1, IL6, TNF-alpha (macrophages)
IFN-gamma (T-cells) |
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Term
| which antibody has the shortest half life? |
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Definition
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Term
| autoantibodies in sjogrens |
|
Definition
Anti-Ro (SS-A)
Anti-La (SS-B)
ANA
rheumatoid factor
SS-A and SS-B are antiribonucleoproteins) |
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Term
|
Definition
| Sjogrens (AKA anti SS-A & SS-B) |
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Term
|
Definition
| anti centromere (C for Centromere & CREST) |
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Term
|
Definition
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Term
|
Definition
|
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Term
|
Definition
| Anti Scl-70 (topoisomerase I) |
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Term
| what is the single most important cytokine in a type 4 hypersensitivity reaction (delayed type hypersensitivity rxn) |
|
Definition
| IFN-gamma secreted by Th1 cells |
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Term
| important cytokine for pathogens such as mycobacterium, listeria, leishmania, and histoplasma |
|
Definition
| IFN-gamma (b.c. they are all intracellular pathogens) |
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|
Term
| hematopoietic colony stimulating factor important in regenerating new myeloid stem cells from bone marrow |
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Definition
|
|
Term
| do T cells mature from thymus cortex to medulla or other way around |
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Definition
| Cortex to medulla (express CD2,3,4,8 in cortex; in medulla, they lose either CD4 or CD8) |
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Term
| what are the most important chemotactic factors for neutrophils |
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Definition
|
|
Term
| which 2 cytokines stimulate endothelial cells to express adhesion molecules |
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Definition
|
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Term
|
Definition
| c-ANCA (anti proteinase 3, PR-3) |
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Term
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Definition
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Term
| marker for drug induced SLE |
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Definition
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Term
| Eosinophilic infiltrate affecting the respiratory tract and necrotizing vasculitis of small to medium sized vessels. |
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Definition
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|
Term
| what can cure thrombocytopenia in wiskott aldrich syndrome? |
|
Definition
| splenectomy, however this predisposes to encapsulated infections & pts need to be on prophylactic antibiotics |
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Term
| gene responsible for x-linked disease w/ thrombocytopenia, eczema, and immunodeficiency |
|
Definition
Wiskott Aldrich Serum Protein
this gene causes reorganization of the cytoskeleton in hematopoietic cells in response to external stimuli |
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Term
|
Definition
AKA verminous pneuomonia
- triad of urticaria, eosinophilia, and asthma
it is a type 1 hypersensitivity reaction, most commonly caused by ascaris (fecal-oral transmission)
other worms commonly involved: strongyloides, ancyclostoma duodenale & necator americanus |
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Term
| pathogenesis of chronic rejection |
|
Definition
T lymphocytes damage endothelial cells over many years
antibodies, immune complexes, slow cellular reaction, recurrences of disease are all responsible, but T-cell mediated damage is most important
- immunosuppressive therapy is directed at T-cells (mycophenolate mofetil) |
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Term
| pathogenesis of acute rejection |
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Definition
| activated T-cells (HLA incompatibility) |
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Term
MHC-I deficiency signs and symptoms
what shouldn't you give to these patients? |
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Definition
- no CD8+ T cells
- susceptibility to intracellular pathogens
- live, attenuated vaccines (i.e. MMR) |
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Term
|
Definition
| chronic lymphocytic leukemia (CD19/20 B-like cells) |
|
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Term
| three components of tissue compatability testing? |
|
Definition
- ABO blood type
- microcytotoxic test (MHC I)
- mixed lymphocyte reaction (MHC II) |
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Term
| which 2 viruses directly infect oligodendrocytes? |
|
Definition
| JC virus & measles (PML & SSPE) |
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Term
| pathogenesis of: subacute onset of proximal muscle weakness, somtimes pain, and elevated creatine kinase in an AIDS pt |
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Definition
| AIDS-associated myopathy: HIV infected macrophages infiltrate nerves & muscle |
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Term
|
Definition
| B cells (CD21 is the receptor for EBV) |
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Term
| initial reservoir for HIV |
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Definition
| follicular dendritic cells (CXCR4); some are inside cells, but most just hang out on the dendritic processes in the lymphoid follicle |
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Term
| in someone who received the S.pneumo vaccine, what would be the initial mechanism of removal if the bacteria got in the blood |
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Definition
| C3b mediated phagocytosis. This is because the vaccine only generates IgM (it's a polysaccharide vaccine that doesn't recruit a T-cell response). IgM would create C3b through classic complement activation and C3b acts as an opsonizing agent for phagocytes in the spleen |
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Term
| what complement deficiency can cause recurrent encapsulated bacterial infections? |
|
Definition
| C3 deficiency (also causes immune complex diseases) |
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Term
|
Definition
type 4 hypersensitivity:
CD4+ Th1 cells react against myelin basic protein --> secrete cytokines (IFN-gamma) --> macrophages destroy myelin |
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Term
| what Th cell response is needed to hold toxo in check? |
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Definition
| Th1 (it's an intracellular protozoan parasite) |
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Term
| how many ABO alleles do we have? |
|
Definition
2 for each (i.e. 2 A, 2B & 2Rh)
this means we can be A+ and be A/-, Rh+/Rh- etc etc |
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|
Term
|
Definition
acetylcholine esterase inhibitor
used to differentiate Myasthenia Gravis from cholenergic crisis:
- if given to a MG pt --> muscle strength improves
- if given to a pt in cholenergic crisis --> muscle strength decreases further (depolarizing block) |
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Term
| x-linked immunodeficiencies |
|
Definition
- brutons agammglobulinemia
- wiskott aldrich
- some cases of SCID |
|
|
Term
| C1 esterase inhibitor deficiency |
|
Definition
| hereditary angioedema (autosomal dominant) |
|
|
Term
| hereditary angioedema pathogenesis |
|
Definition
C1 esterase inhibitor deficiency --> classic complement cascade overactivated (C4b2b3b & vasoactive C3a & C5a) --> capillary permiability & edema of many organs
laryngeal edema can be fatal |
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Term
| this bacterial peptide is a strong chemotactic factor |
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Definition
formyl methionyl peptide
the formylated methionine is the unique starting amino acid in bacterial translation |
|
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Term
|
Definition
product of leukocytes: inhibits viral replication
used in chronic hepatitis B & C and Kaposi's sarcoma |
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Term
|
Definition
produced by fibroblasts & inhibits viral replication
It is used in the treatment of MS |
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Term
| Use IFN-gamma in this immunodeficiency disease |
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Definition
|
|
Term
| transplant from identical twin |
|
Definition
syngenic graft
high rate of success but still requires lifelong immunosupression b.c. of mutations acquired during gestation & development |
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Term
| molecules responsible for strong adhesions of leukocytes to endothelium |
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Definition
LFA-1 (on leukocytes, CD18); ICAM-1 (on endothelium)
these are the most important molecules in diapedesis (CD18 deficiency = leukocyte adhesion deficiency syndrome) |
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Term
|
Definition
| binds fibrinogen, iCb3, & ICAM-1: it promotes movement through extracellular matrix & phagocytosis |
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Term
| receptors important for honing leukocytes to mucosal surfaces? |
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Definition
| MadCAM1 and L-selectin; thought to be important in creating mucosal immune system |
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Term
| P selectins & addressins actions |
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Definition
found on endothelium & platelets: initiate clotting process in area of acute inflammation
they are not involved in movement of phagocytes into areas of inflammation |
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Term
| rag gene function & deficiency |
|
Definition
recombinant activating gene (responsible for VDJ recombination)
deficiency results in a form of SCID |
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Term
| cause of hyper IgM syndrome |
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Definition
|
|
Term
| second signal from APC to Th (CD4+) cell in order to activate it |
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Definition
B7 (APC) & CD28 (Th cell)
the first signal is the antigen on MHCII binding tightly to the TCR |
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Term
C3 convertase in classical & alternative complement pathways
C5 converases |
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Definition
Classic: Ig activates C1-->C2bC4b
alternative: C3b binds bacteria --> C3bBb
- C2bC4bC3b & C3bBbC3b respctively |
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Term
| what is considered HIV positive by western? |
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Definition
positive for at least 2 of three HIV antigens (gp41, gp120, gp24)
if results are inconclusive (?positive for only 1: perform PCR) |
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Term
name the 3 important filarial parasite
how does the body fight them? |
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Definition
Wucheria bancrofti, Onchocerca volvulus, Loa Loa
genus "Brugia" also causes elephantiasis
coats them with IgE |
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Term
| C5-C8 deficiencies get what? |
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Definition
| recurrent Neisseria infections (only pathogen that the body seems to need complement to clear efficiently) |
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Term
|
Definition
| combined immunodeficieny w/ Lupus-like syndrome |
|
|
Term
| C1rs, C1s, C4, or C2 deficiency |
|
Definition
| SLE like syndrome & glomerulonephritis |
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Term
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Definition
|
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Term
|
Definition
|
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Term
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Definition
Direct coombs tests the babies blood: add anti Ig antibodies to agglutinate baby's blood if there are anti-Rh antibodies in the baby's serum (from the mom)
Add Rh+ RBCs to mom's serum (that has the anti-Rh); clumping means there is anti-Rh in the mom's serum and the baby is at risk for EF |
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