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Microbiology - Immunodeficiency
Lectures 22 - 23
30
Microbiology
Professional
09/28/2010

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Term
common immunodeficiency characteristics
Definition
recurrent skin rashes or abscesses

chronic diarrhea

abnormal growth

hepatosplenomegaly


thrombocytopenia

endocrinopathies
Term
leukocyte adhesion deficiency
Definition
the following scenario is descriptive of (blank)

phagocytic cell dysfunction

defective integrin (CD18)

leukocytes can't move from blood to tissue; no diapedesis
Term
chronic granulomatous disease

superoxide

nitroblue tetrazolium dye
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
Term
percentages of immunodeficiency diseases

T cell?

Phagocytic cell?

complement?
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
Term
direct influences

development
Definition
(blank) influences - cause B and T cell immunodeficiencies

are associated with blocks in (blank) of lymphocyte differentiation and function
Term
indirect influences

regulation
Definition
(blank) influences - cause B and T cell immunodeficiencies

are associated with blocks in (blank) of lymphocyte differentiation and function
Term
antibody

B cells

SURFACE Ig POSITIVE CELLS

staphylococcal protein A
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)
Term
staphylococcal protein A

mitosis
Definition
(blank) is a B cell specific mitogen

causes (blank) in a B cell
Term
intracellular

CD3

CD4

CD8

PHYTOHEMAGGLUTININ (PHA
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
Term
Adenosine Deaminase (ADA) Deficiency

Purine Nucleoside Phosphorylase (PNP) Deficiency
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
Term
SCIDs
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!)
Term
X-linked
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
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
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)
Term
x-linked congenital agammaglobulinemia

TYROSINE KINASE (btk)
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!)
Term
puto
Definition
efff you
Term
Selective IgA deficiency
X-linked hyper-IgM syndrome
Activation-induced cytidine deaminase (AID) Deficiency

SELECTIVE IgA DEFICIENCY

older

equal

plasma
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
Term
selective IgA deficiency
Definition
most common immunodeficiency (1 in 400)

vast majority remain asymptomatic
Term
CD40 ligand

isotype switching

IgM
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)
Term
Activation-induced cytidine deaminase (AID) Deficiency
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
Term
DiGeorge's Syndrome

thymic aplasia

thymus

no calcium, high phosphorus, muscle tetany
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)
Term
mucocutaneous candidiasis

yeast
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
Term
acquired immunodeficiency syndrome (AIDS)
Definition
(blank) is transmitted by HIV virus

know modes of transmission and certain behaviors that are high risk
Term
CD4+

Gp120
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
Term
CCR5 and CXCR4
Definition
chemokine receptors (Ts and macs for CCR5 and Ts only for CXCR4)

used as co-receptors to facilitate HIV entry into immune cells
Term
Kaposi's Sarcoma
Definition
cancer that increases in incidence among HIV patients
Term
tools for monitoring course of HIV infection
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)
Term
what is normal level of CD4 T cells

what about in asymptomatic HIV patients?

full blown AIDS
Definition
1200/micro liter

800/micro liter

less than 200/micro liter
Term
describe chain reaction that happens once CD4+ cells are destroyed in an HIV patient's immune system
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)
Term
bare-leukocyte syndrome

class I or class II MHC molecules

cell-mediated immunity

humoral immunity

TAP genes

CD8+
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
Term
RAG deficiency syndrome

Recombination Activation Genes

RAG1/RAG2
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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