Term
| common immunodeficiency characteristics |
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Definition
recurrent skin rashes or abscesses
chronic diarrhea
abnormal growth
hepatosplenomegaly
thrombocytopenia
endocrinopathies |
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Term
| leukocyte adhesion deficiency |
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Definition
the following scenario is descriptive of (blank)
phagocytic cell dysfunction
defective integrin (CD18)
leukocytes can't move from blood to tissue; no diapedesis |
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Term
chronic granulomatous disease
superoxide
nitroblue tetrazolium dye |
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Definition
the following describes (blank) disease
can't make (blank)
affects the ability of phagocytes to destroy ingested bacteria; think of oxidative respiratory burst
inability to reduce (blank) is common lab test of this |
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Term
percentages of immunodeficiency diseases
T cell?
Phagocytic cell?
complement? |
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Definition
50% B cell associated
30-40% T cell
15% Phagocytic cell
2-4% complement
these values represent the percentages of (blank) diseases due to their respective cell types |
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Term
direct influences
development |
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Definition
(blank) influences - cause B and T cell immunodeficiencies
are associated with blocks in (blank) of lymphocyte differentiation and function |
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Term
indirect influences
regulation |
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Definition
(blank) influences - cause B and T cell immunodeficiencies
are associated with blocks in (blank) of lymphocyte differentiation and function |
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Term
antibody
B cells
SURFACE Ig POSITIVE CELLS
staphylococcal protein A |
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Definition
the following describes the sign posts of a deficiency in antibody-mediated (humoral) immunity:
many (extra or intra cellular?) infections
reduced serum (blank) levels
reduced circulating (blank) cells; decreased percent of (blank) [(blank) cells should be at abt 20-30% of lymphocytes]
reduced FUNCTIONAL (blank) cells; responsiveness to (blank) cell specific mitogens such as (blank) |
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Term
staphylococcal protein A
mitosis |
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Definition
(blank) is a B cell specific mitogen
causes (blank) in a B cell |
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Term
intracellular
CD3
CD4
CD8
PHYTOHEMAGGLUTININ (PHA |
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Definition
the following describes how you can spot cell-mediated immunity immunodeficiency:
frequent viral, fungal, and (blank) bacterial infections
reduced circulating (blank) cells; such as those that express (blank) [all mature (blank) cells do!!], (blank) [which is characteristic of helpers], or (blank) [characteristic of killers]
reduced FUNCTIONAL (blank) cells, which should respond to (blank), a mitogen |
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Term
Adenosine Deaminase (ADA) Deficiency
Purine Nucleoside Phosphorylase (PNP) Deficiency |
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Definition
50% of autosomal SCIDs is caused by deficiency of (blank) or (blank)
both these enzymes involved in nucleotide metabolism
In SCIDs, progenitors of lymphocytes don't have working methods to get rid of toxic intermediaries and thus die... = basically no immune system |
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Term
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Definition
the following describe the 3 main autosomal origins of (blank)
1. defect in ADA or PNP (nucleotide metabolism)
2. Bare-leukocyte syndrome (no MHCs on APCs)
3. RAG deficiencies (can't recombinate to make antigen receptors!) |
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Term
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Definition
the following describes (blank) type of SCID
defective common gamma chain of cytokine receptors
can't receive important cytokines
IL-2, 4, 7, 9 and 15
IL-7 most important = used in lymphocyte development |
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Term
agammaglobulinemias
X-linked congenital agammaglobulinemia
and
Common Variable Immunodeficiency (CVI)
Selective IgA deficiency
X-linked hyper-IgM syndrome
Activation-induced cytidine deaminase (AID) Deficiency |
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Definition
(blank) describes scenarios in which the patient displays deficiencies in antibody production
it is the umbrella term for anything related to deficiency with B cell branch
further divided into (blank) and (blank)
(blank) can further be divided into 3 sub categories, (blank), (blank) and (blank) |
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Term
x-linked congenital agammaglobulinemia
TYROSINE KINASE (btk) |
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Definition
the following describes x-linked congenital agammaglobulinemia:
B cells stuck at the (blank) stage
DEFECTIVE (blank)
(above blank) necessary for appropriate V to DJ joining [gene rearrangements] leading to proper antigen receptor expression
primarily a disease of young males (x-linked!) |
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Term
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Definition
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Term
Selective IgA deficiency
X-linked hyper-IgM syndrome
Activation-induced cytidine deaminase (AID) Deficiency
SELECTIVE IgA DEFICIENCY
older
equal
plasma |
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Definition
the following describes common variable immunodeficiencies (CVI): [remember this represents a subset of agammaglobulinemias apart from X-linked congenital agammaglobulinemia!]
CVI can further be divided into 3 subdivisions (blank), (blank), and (blank)
selective isotype deficiencies of any isotype can be observed (ex: no IgE or no IgD)
most common of these isotype deficiencies is (blank)
affects what general age group of patient population? (blank)
frequencies of affected males and females are (blank)
B cells (but no (blank) cells) often present
mechanisms for these syndromes are complex and unclear |
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Term
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Definition
most common immunodeficiency (1 in 400)
vast majority remain asymptomatic |
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Term
CD40 ligand
isotype switching
IgM |
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Definition
the following describes X-linked hyper-IgM syndrome:
defective (blank) on activated T cells; which is used to activate B cells
thus, cannot stimulate B cells to differentiate to IgG = no (blank)
means lots and lots of (blank) |
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Term
| Activation-induced cytidine deaminase (AID) Deficiency |
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Definition
| (blank) is a type of condition of CVI [agammaglobulinemia] in which the patient demonstrates inability to isotype class switch and B cells cannot reach affinity maturation |
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Term
DiGeorge's Syndrome
thymic aplasia
thymus
no calcium, high phosphorus, muscle tetany |
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Definition
(blank) - developmental defects affecting all tissues in the upper chest cavity, striking set of facial features
congenital (blank) (blank) [(blank) deformed or absent]
uneducated pre-T cells present
no (blank), high (blank), muscle (blank) |
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Term
mucocutaneous candidiasis
yeast |
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Definition
the following describes (blank) (blank)
T cells of cell-mediated immunity branch don't work (CTLs, TH1 and TH17); caused by (blank) infection
simple (blank) infections can disseminate systemically and become life-threatening |
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Term
| acquired immunodeficiency syndrome (AIDS) |
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Definition
(blank) is transmitted by HIV virus
know modes of transmission and certain behaviors that are high risk |
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Term
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Definition
what does HIV infect?
cells of immune system, especially (blank) cells
(blank) of HIV binds to CD4+
chemokine receptors CCR5 (Ts and Macs) and CXCR4 (only Ts) are used as "co-receptors" for the virus |
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Term
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Definition
chemokine receptors (Ts and macs for CCR5 and Ts only for CXCR4)
used as co-receptors to facilitate HIV entry into immune cells |
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Term
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Definition
| cancer that increases in incidence among HIV patients |
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Term
| tools for monitoring course of HIV infection |
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Definition
screening for HIV seropositivity (ELISA assay)
-danger of false positives (confirm with WB)
-danger of false negatives (early diagnosis difficult)
tests for presence of HIV genome using PCR
tests for circulating CD4+ cells (immunofluorescence with monoclonal antibodies) |
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Term
what is normal level of CD4 T cells
what about in asymptomatic HIV patients?
full blown AIDS |
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Definition
1200/micro liter
800/micro liter
less than 200/micro liter |
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Term
| describe chain reaction that happens once CD4+ cells are destroyed in an HIV patient's immune system |
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Definition
CD4+ T lymphocytes consist of TH1 and TH2 cells and are responsible for activating inflammatory/cell-mediated (TH1) and humoral (TH2) responses.
TH1 subset (IL-2, IFN-gamma, TNF-alpha)
TH2 subset (IL-4, IL-5, IL-10) |
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Term
bare-leukocyte syndrome
class I or class II MHC molecules
cell-mediated immunity
humoral immunity
TAP genes
CD8+ |
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Definition
the following describes (blank), one of three autosomal origins of SCIDs:
a family of disorders involving deficient expression or function of (blank) or (blank) (blank) molecules
Direct interference with (blank)-(blank) (blank)
Indirect interference with (blank) (blank)
one variation involved mutated (blank) genes that code for protein that pumps protein fragments into ER; resulting in impaired antigen presentation to (blank) T cells |
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Term
RAG deficiency syndrome
Recombination Activation Genes
RAG1/RAG2 |
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Definition
the following describes (blank), one of three autosomal origins of SCIDs:
it involves the impaired expression of (blank) genes
these genes code for proteins/enzymes called (blank), which are involved in genetic rearrangements in B and T cells
without these proteins, antigen receptors cannot be produced - nonfunctional immune system |
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