IMMUNODEFICIENCY

Question 1

Two major kinds of defense have evolved that counter threats:

–________ immunity and _______ immunity

Answer 1

Innate; acquired

Question 2

Innate immunity
– Is present _______________ and is effective from ____________

– Involves (specific or nonspecific?) responses to pathogens

Answer 2

before any exposure to pathogens

the time of birth

nonspecific

Question 3

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

Answer 3

adaptive

exposure to inducing agents

very specific

Question 4

Natural Killer Cells

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

Answer 4

virus; cancer

apoptosis

Question 5

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

Answer 5

Active

Question 6

In acquired immunity, ________ provide specific defenses against infection

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

Answer 6

lymphocytes

second

lymphocytes

Question 7

An antigen is any foreign molecule

•That is specifically recognized by _________ and ______________

Answer 7

lymphocytes; elicits a response from them

Question 8

A lymphocyte actually recognizes and binds

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

Answer 8

epitope

Question 9

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

Answer 9

100,000

Question 10

B Cell Receptors for Antigens

B cell receptors

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

Answer 10

Specific

membrane antibodies or membrane immunoglobulins

Question 11

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.

Answer 11

antibodies; humoral

bone marrow; spleen

naive mature

Question 12

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

Answer 12

antigen; receptors

plasma; antibodies

long; memory

Question 13

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

Answer 13

B; mutate the genes

produce antibodies

bind to

Question 14

Somatic hypermutation occurs in T- cell receptor genes

T/F

Answer 14

F

Somatic hypermutation does not occur in T- cell receptor genesh

Question 15

Each T cell receptor
– Consists of ______ different polypeptide chains

Answer 15

two

Question 16

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

Answer 16

Small

MHC

Question 17

MHC molecules

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

Answer 17

major histocompatibility complex

Question 18

Infected cells produce MHC molecules

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

Answer 18

antigen fragments

Cell surface

antigen presentation

Question 19

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

Answer 19

macrophages, dendritic cells and В cells

Question 20

After antigen presentation :

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

Answer 20

antigen fragment

cell’s surface

Question 21

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

Answer 21

Different

Question 22

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

It Display ________ antigens to _________ cells

Answer 22

almost all nucleated

peptide; cytotoxic T

Question 23

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

– it Display antigens to ___________ cells

Answer 23

dendritic cells, macrophages, and B cells

helper T

Question 24

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

Results in __________ disease

Answer 24

one or more elements of the immune system

immunodeficiency

Question 25

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

Answer 25

Primary (congenital) – Secondary (acquired)

Question 26

PRIMARY IMMUNODEFICIENCIES Primary immunodeficiencies are _______ defects of the immune system These defects may be in the _______ or __________ immune mechanisms

Answer 26

inherited specific or nonspecific

Question 27

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

Answer 27

site of lesion

Question 28

B CELL DEFICIENCY List 6

Answer 28

Common variable immundeficiency X liked agammaglobuinemia IgA deficiency IgG subclass deficiency Immunodeficiency with increased IgM Transient hypogammaglobulinaemia of infancy

Question 29

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

Answer 29

early maturation of B cells Few or no Very small Reduced; absence G; pyogenic

Question 30

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

Answer 30

A immune complex 20; pyogenic terminal differentiation

Question 31

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 __________

Answer 31

IgA and IgG Large ; >200 pyogenic iv gamma globulin M; isotype switching

Question 32

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 ___________

Answer 32

T cell signaling agammaglobulinemia; 2nd or 3rd viral; Pyogenic are not functioning defective; receive signaling from T lymphocytes

Question 33

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 ________________

Answer 33

G; 36 months 3 months Normal help of T lymphocytes

Question 34

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

Answer 34

Most congenital thymic aplasia/hypoplasia hypo; heart; fish development of fetus during 6th-10th week of gestation parathyroid, thymus, lips, ears and aortic arch

Question 35

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 ___________

Answer 35

coordination of movement dilation of small blood vessels of the facial reduced to various degrees. normal to low.

Question 36

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

Answer 36

Normal to low reduced reduced High ; leukemia 14; T cell receptor ; Ig heavy chain

Question 37

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.

Answer 37

progressively reduces ; normal blood levels reduced; normal elevated; elevated eczema; polysaccharide pyogenic

Question 38

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.

Answer 38

Bare leukocyte MHC class II ; transactivator class- II MHC ; fewer transport associated ; class-I MHC CD8+ T

Question 39

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.

Answer 39

Low ; neutrophil; three weeks A week; susceptible to infection poor regulation

Question 40

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 ________.

Answer 40

lymphadenopathy, hepato-splenomegaly and chronic draining lymph nodes. NADPH oxidase respiratory burst

Question 41

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.

Answer 41

CR3; CD11; CD18; C3b opsonin. integrin ; LFA-1 or mac-1 CD11a or CD11b diapedesis

Question 42

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

Answer 42

intracellular killing and chemotactic movement phagosome and lysosome fusion proteinase; normal NK cell platelet and neurological

Question 43

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 ________

Answer 43

susceptibility to infections. C3 deficiency low C3 synthesis factor I or factor H.

Question 44

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.

Answer 44

50; x-linked; autosomal an absence of T cell and B cell immunity absence (or very low numbers) of circulating T and B lymphocytes.

Question 45

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 ______

Answer 45

gamma IL-2 IL-4, -7, -11 and 15 proliferation and/or differentiation.

Question 46

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

Answer 46

adenosine deaminase (ADA) purine nucleoside phosphorylase dATP or dGTP lymphoid stem

Question 47

Diagnosis of SCID Is based on _______ of T and B cells and _______ measurement. Severe combined immunodeficiency can be treated with ________________

Answer 47

enumeration immunoglobulin bone marrow transplant

Question 48

IMMUNODEFICIENCY CAUSED BY DRUGS (IATROGENIC) 1. CORTICOSTEROIDS Cause changes in _____________ Depletion of _____ cells Monocytopenia Decreased in circulating _______ and _______ Inhibition of _________ and ________ Inhibit cytokine synthesis

Answer 48

circulating leukocytes CD4 eosinophils and basophils T cell activation and B cell maturation

Question 49

IMMUNODEFICIENCY CAUSED BY DRUGS (IATROGENIC) METHOTREXATE A ___________ Of _______ Blocks ________________ pathways essential for __________ Prolonged use for treatment reduces _____________

Answer 49

Structural analogue of folic acid folic acid dependent synthetic DNA synthesis immunoglobulin synthesis

Question 50

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

Answer 50

T cell signaling immunophilins signal transduction IL 2

Question 51

IONIZING RADIATION ___-rays or _____ rays used in tumour treatment destroys lymphoid tissues also. Ionizing radiation generally kills ______ cells in a _______ manner but also affects ______________

Answer 51

X gamma proliferating highly selective resting lymphocytes

Question 52

INFECTIONS – Results in more or less _____________________ HIV pathogenesis is a direct consequence of its __________ of immune responsiveness affecting especially _________-cells

Answer 52

general immunosuppression. severe depression CD4 T

Question 53

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

Answer 53

children Enteropathies