IMMUNODEFICIENCY Flashcards

1
Q

Two major kinds of defense have evolved that counter threats:

–________ immunity and _______ immunity

A

Innate; acquired

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2
Q

Innate immunity
– Is present _______________ and is effective from ____________

– Involves (specific or nonspecific?) responses to pathogens

A

before any exposure to pathogens

the time of birth

nonspecific

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3
Q

Acquired immunity, also called ______ immunity

– Develops only after ____________________ such as microbes, toxins, or other foreign substances
– Involves a ( specific or non-specific ?) response to pathogens

A

adaptive

exposure to inducing agents

very specific

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4
Q

Natural Killer Cells

Natural killer (NK) cells
– Patrol the body and attack ______-infected body cells and _____ cells
– Trigger ______ in the cells they attack

A

virus; cancer

apoptosis

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5
Q

The lymphatic system
– Plays a/an (active or passive?) role in defending the body from pathogens

A

Active

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6
Q

In acquired immunity, ________ provide specific defenses against infection

Acquired immunity
– Is the body’s ______ major kind of defense
– Involves the activity of ___________

A

lymphocytes

second

lymphocytes

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7
Q

An antigen is any foreign molecule

•That is specifically recognized by _________ and ______________

A

lymphocytes; elicits a response from them

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8
Q

A lymphocyte actually recognizes and binds

•To just a small, accessible portion of the antigen called an _______

A

epitope

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9
Q

Antigen Recognition by Lymphocytes

Two main types of lymphocytes
– B lymphocytes (B cells) and T lymphocytes Which circulate through the blood

The plasma membranes of both B cells and T cells
– Have about _______ antigen receptor that all recognize the same epitope

A

100,000

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10
Q

B Cell Receptors for Antigens

B cell receptors

Bind to (specific or non-specific?) , intact antigens
Are often called _________ or ___________

A

Specific

membrane antibodies or membrane immunoglobulins

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11
Q

B lymphocytes or B cells produce _______ involved in ______ immunity.

B cells are produced in the _______, where the initial stages of maturation occur, and travel to the _____ for final steps of maturation into __________ B cells.

A

antibodies; humoral

bone marrow; spleen

naive mature

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12
Q

B-cells are activated by the binding of ______ to ______ on its cell surface which causes the cell to divide and proliferate.

Some stimulated B-cells become ______ cells, which secrete _______.

Others become ____-lived ____ B-cells which can be stimulated at a later time to differentiate into plasma cells

A

antigen; receptors

plasma; antibodies

long; memory

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13
Q

Somatic hypermutation is a process that allows ____ cells to _________ that they use to produce antibodies.

This enables the B cells to _________ that are better able to _______ bacteria, viruses and other infections

A

B; mutate the genes

produce antibodies

bind to

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14
Q

Somatic hypermutation occurs in T- cell receptor genes

T/F

A

F

Somatic hypermutation does not occur in T- cell receptor genesh

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15
Q

Each T cell receptor
– Consists of ______ different polypeptide chains

A

two

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16
Q

T cells bind to (small or long?) fragments of antigens That are bound to normal cell-surface proteins called ______ molecules

A

Small

MHC

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17
Q

MHC molecules

MHC molecules
Are encoded by a family of genes called the ______________

A

major histocompatibility complex

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18
Q

Infected cells produce MHC molecules

– Which bind to _____________ and then are transported to the ______ in a process called __________

A

antigen fragments

Cell surface

antigen presentation

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19
Q

There are three types of antigen presenting cells in the body: __________,__________,__________ .

A

macrophages, dendritic cells and В cells

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20
Q

After antigen presentation :

A nearby T cell
– Can then detect the _______ displayed on the _______ of APC

A

antigen fragment

cell’s surface

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21
Q

Peptide antigens are handled by (the same or different?) classes of MHC molecules

A

Different

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22
Q

Class I MHC molecules, found on ______________ cells of the body

It Display ________ antigens to _________ cells

A

almost all nucleated

peptide; cytotoxic T

23
Q

Class II MHC molecules, located mainly on _______,_______, and ________

– it Display antigens to ___________ cells

A

dendritic cells, macrophages, and B cells

helper T

24
Q

IMMUNODEFICIENCY
It is the absence or failure of normal function of _________________

Results in __________ disease

A

one or more elements of the immune system

immunodeficiency

25
Q

Immunodeficiency

Can be classified as
–_________ (________)
– _________ (_________)

A

Primary (congenital)
– Secondary (acquired)

26
Q

PRIMARY IMMUNODEFICIENCIES

Primary immunodeficiencies are _______ defects of the immune system

These defects may be in the _______ or __________ immune mechanisms

A

inherited

specific or nonspecific

27
Q

PRIMARY IMMUNODEFICIENCIES

They are classified on the basis of the ____________ in the developmental or differentiation pathway of the immune system

A

site of lesion

28
Q

B CELL DEFICIENCY

List 6

A

Common variable immundeficiency

X liked agammaglobuinemia

IgA deficiency
IgG subclass deficiency

Immunodeficiency with increased IgM

Transient hypogammaglobulinaemia of infancy

29
Q

1- X-linked agammaglobulinaemia

In X-LA, __________________ fails

__________ B cells in blood

____________ lymph nodes and tonsils

_______ level or ______ of plasma immunoglobulins

Small amount of Ig___ in early age
Recurrent _____ infection

A

early maturation of B cells

Few or no

Very small

Reduced; absence

G; pyogenic

30
Q

IgA and IgG subclass defeciency

Ig____ deficiency is most common

Patients tend to develop _______ disease

About ___% lack IgG2and IgG4

Susceptible to _______ infection

Result from failure in _______________ of B cells

A

A

immune complex

20; pyogenic

terminal differentiation

31
Q

Immunodfeiciency with increased IgM (HIgM)

Results in patients with ___________ deficiency

Production of (small or large?) amount of IgM=_______ mg/dl of polyclonal IgM

Susceptible to _______ infection

Treatment by _________

Formation of Ig___ to neutrophils, platelets and other blood components

Due to inability of B cells to __________

A

IgA and IgG

Large ; >200

pyogenic

iv gamma globulin

M; isotype switching

32
Q

Common Variable Immunodeficiency (CVID)

Defect in ____________ to B cells

Acquired _______ in the _____ or _____ decade of life

May follow ____ infection

Susceptibile to _____ infection

80% of patients have B cells that ________

B cells are not _______. They just fail to ___________

A

T cell signaling

agammaglobulinemia; 2nd or 3rd

viral; Pyogenic

are not functioning

defective; receive signaling from T lymphocytes

33
Q

Hypogamaglobulinaemia of infancy

Due to delay in in Ig___ synthesis approximately up to ______

In normal infants synthesis begins at _________

( Normal or Abnormal?) B lymphocytes

Probably lack ________________

A

G; 36 months

3 months

Normal

help of T lymphocytes

34
Q

DISORDERS of T CELLS
A. DiGeorge’s syndrome:

It the (most or least?) understood T-cell immunodeficiency

Also known as _______/________

Associated with ____parathyroidism, congenital _____ disease, ____ shaped mouth.

Defects results from abnormal ____________________ during ____________ when ———,——,——-,——, and _____ are being formed

A

Most

congenital thymic aplasia/hypoplasia

hypo; heart; fish

development of fetus during 6th-10th week of gestation

parathyroid, thymus, lips, ears and aortic arch

35
Q

T cell deficiencies with variable degrees of B cell deficiency
1- Ataxia-telangiectasia:

Associated with a lack of ______________ (ataxis) and ___________ of the __________ area (telangiectasis).

T-cells and their functions are _____________

B cell numbers and IgM concentrations are ___________

A

coordination of movement

dilation of small blood vessels of the facial

reduced to various degrees.

normal to low.

36
Q

Ataxia-telangiectasia:

IgM concentrations are ___________
IgG is often _______
IgA is considerably ______ (in 70% of the cases).
There is a (low or high?) incidence of malignancy, particularly _______ in these patients.

There is defect in chromosome ____ at the site of ___________ and _______ genes

A

Normal to low

reduced

reduced

High ; leukemia

14; T cell receptor ; Ig heavy chain

37
Q

T cell deficiencies with variable degrees of B cell deficiency

Wiskott-Aldrich syndrome:

T cell function _________ despite __________

IgM concentrations are ______

IgG levels may be _______

IgA levels are _______.

IgE levels are ________

Boys with this syndrome develop severe _____________.

They respond poorly to ______ antigens and are prone to _______ infection.

A

progressively reduces ; normal blood levels

reduced; normal

elevated; elevated

eczema; polysaccharide

pyogenic

38
Q

MHC DEFICIENCY
(__________ syndrome):

Due to defect in the ________ ———- (CIITA) protein gene, which results in a lack of ________ molecule on APC.

Patients have ——— CD4 cells and are infection prone

There are also individuals who have a defect in their __________ protein (TAP) gene and hence do not express the ________ molecules and consequently are deficient in _______ cells.

A

Bare leukocyte

MHC class II ; transactivator

class- II MHC ; fewer

transport associated ; class-I MHC

CD8+ T

39
Q

Defects of the phagocytic system

1- Cyclic neutropenia:
It is marked by (low or high?) numbers of circulating _______ approximately every _______.

The neutropenia lasts about _____ during which the patients are ____________.

The defect appears to be due to _________ of neutrophil production.

A

Low ; neutrophil; three weeks

A week; susceptible to infection

poor regulation

40
Q

Defects of the phagocytic system

Chronic granulomatous disease (CGD):

CGD is characterized by marked _______,___________, and __________

In majority of patients with CGD, the deficiency is due to a defect in ________ that participate in phagocytic ________.

A

lymphadenopathy, hepato-splenomegaly and chronic draining lymph nodes.

NADPH oxidase

respiratory burst

41
Q

Defects of the phagocytic system

Leukocyte Adhesion Deficiency:

Leukocytes lack the complement receptor _____ due to a defect in _____ or _____ peptides and consequently they cannot respond to _________.

Alternatively there may a defect in _____ molecules, ________ or _______ arising from defective ______ or _______ peptides, respectively.

These molecules are involved in ______ and hence defective neutrophils cannot respond effectively to chemotactic signals.

A

CR3; CD11; CD18; C3b opsonin.

integrin ; LFA-1 or mac-1

CD11a or CD11b

diapedesis

42
Q

Defects of the phagocytic system

Chediak-Higashi syndrome:

This syndrome is marked by reduced (slower rate) ________ and _______ accompanied by inability of _______________________ and _____ deficiency.

Respiratory burst is _______.

Associated with ______ defect, _______ and _________ disorders

A

intracellular killing and chemotactic movement

phagosome and lysosome fusion

proteinase; normal

NK cell

platelet and neurological

43
Q

Disorders of complement system:

Complement abnormalities also lead to increased ______________

The most serious among these is the __________ which may arise from _____________ or deficiency in _________ or ________

A

susceptibility to infections.

C3 deficiency

low C3 synthesis

factor I or factor H.

44
Q

Disorders of complement system

SEVERE COMBINED IMMUNODEFICENCY

In about ____% of SCID patients the immunodeficiency is ______ whereas in the other half the deficiency is ________.

They are both characterized by ______________________________ and __________ of __________________

Patients with SCID are susceptible to a variety of bacterial, viral, mycotic and protozoan infections.

A

50; x-linked; autosomal

an absence of T cell and B cell immunity

absence (or very low numbers) of circulating T and B lymphocytes.

45
Q

Disorders of complement system

The x-linked SCID is due to a defect in _______-chain of ________ also shared by ____,_____,_____, and ______, all involved in lymphocyte _______ and/or ______

A

gamma

IL-2

IL-4, -7, -11 and 15

proliferation and/or differentiation.

46
Q

Disorders of complement system

The autosomal SCIDs arise primarily from defects in ______________ or ____________ (PNP) genes which results is accumulation of _______ or ______ , respectively, and cause toxicity to ___________ cells

A

adenosine deaminase (ADA)

purine nucleoside phosphorylase

dATP or dGTP

lymphoid stem

47
Q

Diagnosis of SCID

Is based on _______ of T and B cells and _______ measurement.

Severe combined immunodeficiency can be treated with ________________

A

enumeration

immunoglobulin

bone marrow transplant

48
Q

IMMUNODEFICIENCY CAUSED BY DRUGS (IATROGENIC)

  1. CORTICOSTEROIDS
    Cause changes in _____________

Depletion of _____ cells
Monocytopenia

Decreased in circulating _______ and _______

Inhibition of _________ and ________ Inhibit cytokine synthesis

A

circulating leukocytes

CD4

eosinophils and basophils

T cell activation and B cell maturation

49
Q

IMMUNODEFICIENCY CAUSED BY DRUGS (IATROGENIC)

METHOTREXATE
A ___________ Of _______

Blocks ________________ pathways essential for __________

Prolonged use for treatment reduces _____________

A

Structural analogue of folic acid

folic acid dependent synthetic

DNA synthesis

immunoglobulin synthesis

50
Q

IMMUNODEFICIENCY CAUSED BY DRUGS (IATROGENIC)

CYCOLOSPORIN

Have severe effects on __________________ and functions

It binds to _______ which are believed to have a critical role in _______

Also inhibit ____ dependent signal transduction

A

T cell signaling

immunophilins

signal transduction

IL 2

51
Q

IONIZING RADIATION

___-rays or _____ rays used in tumour treatment destroys lymphoid tissues also.

Ionizing radiation generally kills ______ cells in a _______ manner but also affects ______________

A

X

gamma

proliferating

highly selective

resting lymphocytes

52
Q

INFECTIONS

– Results in more or less _____________________

HIV pathogenesis is a direct consequence of its __________ of immune responsiveness affecting especially _________-cells

A

general immunosuppression.

severe depression

CD4 T

53
Q

Malnutritoion
Includes
– Starvation especially in (children or adults?)
–________ causing loss of vital nutrients for building the bodies immune system

A

children

Enteropathies