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House AMP > Immunopathology 3 > Flashcards

Immunopathology 3 Flashcards

1
Q

IMMUNODEFICIENCY DISEASES INTRODUCTION

Congenital B-cell defects are clinically conspicuous because of the reduced resistance to ______ infection that they involve.
In contrast, primary T-cell defects leave the patient unprotected against ____________

A

bacterial

viruses and fungi.

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2
Q

Patients with a combined B cell and T-cell deficiency are poor candidates for survival.

T/F

A

T

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3
Q

Among the acquired immunodeficiency disorders, gammopathy due to ____________ and AIDS due to __________ warrant special mention.

A

neoplastic proliferation of a plasma cell clone

infection with the human immunodeficiency virus (HIV)

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4
Q

Immunodeficiencies can be divided into the primary immunodeficiency disorders, which are almost always _______ determined

And secondary immunodeficiency states, which may arise as complications of _______ factors

A

genetically

External factors

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5
Q

Most primary immunodeficiency diseases are genetically determined and affect only INNATE IMMUNITY

T/F

A

F

Both INNATE IMMUNITY ACQUIRED IMMUNITY

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6
Q

Most primary immunodeficiencies manifest themselves in _____, between _____ and ____ of life, and they are detected because _____________________

A

infancy; 6 months and 2 years

the affected infants are susceptible to recurrent infections.

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7
Q

PRIMARY IMMUNODEFICIENCY

X-Linked Agammaglobulinemia (____
Agammaglobulinemia)

Due to ______

A

Bruton’s

disordered B-cell maturation

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8
Q

In Bruton’s Agammaglobulinemia

Naïve b cells can mature to plasma cells

T/F

A

F

Cannot

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9
Q

Bruton’s Agammaglobulinemia

Caused by mutations in a _______, called __________;

the gene that encodes it is located on the ____ arm of the ___ chromosome at ____.___.

A

cytoplasmic tyrosine kinase; Bruton tyrosine kinase (Btk)

long; X

Xq21.22

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10
Q

Bruton’s Agammaglobulinemia

The disease usually does not become apparent until about _____ of age, as _______

A

6 months

maternal immunoglobulins are depleted.

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11
Q

Bruton’s Agammaglobulinemia

Recurrent ____s infection (________)

A

BEG

bacterial IgG, enteroviruses IgA, giardia

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12
Q

Beware of live attenuated (eg polio) vaccines in patients suffering from Bruton’s Agammaglobulinemia

T/F

A

T

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13
Q

Bruton’s Agammaglobulinemia

Recurrent BEG infection

B Causative Organisms
•———-,———,———

enteroviruses, such as _____,_____,______

A

Haemophilus influenzae
Streptococcus pneumoniae, or
Staphylococcus aureus.

echovirus, poliovirus, and coxsackievirus

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14
Q

In Bruton’s Agammaglobulinemia

•B cells are ______________ in the circulation, and the serum levels of all classes of immunoglobulins are _______.

•Pre-B cells, are found in _____ numbers in the bone marrow.

• Germinal centers of ____,______,______, and ___ are underdeveloped.

• Plasma cells are (present or absent?) throughout the body.

• T cell–mediated reactions are ____.

A

absent or markedly decreased ; depressed

normal

lymph nodes, Peyer’s patches, the appendix, and tonsils

Absent ; normal

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15
Q

Autoimmune diseases, such as _______ and ______, occur with increased frequency, in as many as 35% of individuals with X-linked agammaglobulinemia disease, which is paradoxical ___________

A

arthritis and dermatomyositis

in the presence of an immune deficiency

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16
Q

The treatment of X-linked agammaglobulinemia is ___________

A

replacement therapy with immunoglobulins.

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17
Q

Common Variable Immunodeficiency

(Well or Poorly ?) defined entity

The feature common to all patients is _______, generally affecting all the ________ but ____

A

Poorly

hypogammaglobulinemia

antibody classes

sometimes only IgG.

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18
Q

Relatives of Common Variable Immunodeficiency patients have a high incidence of __________

A

selective IgA deficiency

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19
Q

In contrast to X-linked agammaglobulinemia, most individuals with common variable immunodeficiency have ________ numbers of B cells in the blood and lymphoid tissues.
These B cells, however, are _________

A

normal or near-normal

not able to differentiate into plasma cells.

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20
Q

In contrast to X-linked agammaglobulinemia, common variable immunodeficiency affects (male or female?) , and the onset of symptoms is in ______ or _____

A

both sexes equally

childhood or adolescence

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21
Q

common variable immunodeficiency

Histologically the B-cell areas of the lymphoid tissues (i.e., lymphoid follicles in nodes, spleen, and gut) are _____.

A

hyperplastic

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22
Q

Isolated IgA Deficiency

Most common in the _____ world. It is far less common in _______

A

westerns; blacks and Asians

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23
Q

Isolated IgA Deficiency

Characterized by _____ levels of _________

It may be familial, or acquired in association with toxoplasmosis, measles, or some other viral infection

A

extremely low

both serum and secretory IgA

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24
Q

The basic defect in isolated IgA deficiency is ______________

A

impaired differentiation of naive B lymphocytes to IgA-producing cells

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25
Q

Most individuals with isolated IgA deficiency disease are symptomatic.

T/F

A

F

asymptomatic

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26
Q

Isolated IgA deficiency

Because IgA is the major Ig in ________ , ______ are weakened, and infections occur in the ______,____, and ——- .

A

external secretions

mucosal defenses

respiratory, gastrointestinal, and urogenital tracts

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27
Q

Celiac dx is associated with ___ deficiency

A

IgA

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28
Q

IgA-deficient patients have a high frequency of respiratory tract allergy and a variety of autoimmune diseases, particularly _____ and ______

A

SLE and rheumatoid arthritis.

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29
Q

When transfused with blood containing normal IgA, some of these isolated IgA deficient patients develop _____________ reactions, because ________

A

severe, even fatal, anaphylactic

the IgA behaves like a foreign antigen (since the patients do not produce it and are not tolerant to it)

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30
Q

Hyper-IgM Syndrome

affected patients make ___ antibodies but are deficient in their ability to produce ________ antibodies

A

IgM; IgG, IgA, and IgE

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31
Q

Approximately 70% of individuals with hyper-IgM syndrome have the _____ form of the disease, caused by mutations in the gene encoding ______ located on Xq26. Or ___________.

A

X-linked

CD40L

CD40 receptor

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32
Q

Hyper-IgM Syndrome
Remember that Bcells can be activated to plasma cells in 2 ways-
1)_______ on the surface of naïve Bcells to ___________

2)_____ Antigen and presenting through ______ to T helper cells with _____ as 2nd stimulus. Activated CD4+ produce _____ and ____ which cause heavy chain class switch and production of Ig E, Ig G, Ig A

A

IgM receptors; IgM producing plasma cells

Internalising; MHC class II; CD40

IL4 and IL5

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33
Q

Hyper IgM syndrome

In the remaining persons the disease is inherited in an autosomal recessive pattern.

Most of these patients have mutations in the gene encoding _____ or the enzyme called ________

A

CD40

activation-induced deaminase

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34
Q

Hyper IgM syndrome

The serum of persons with this syndrome contains ________ levels of IgM but ______ IgA or IgE and ______ levels of IgG.

The number of B and T cells is ______.

A

normal or elevated

no; extremely low

normal

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35
Q

In hyper IgM syndrome

Many of the IgM antibodies react with elements of blood, giving rise to _______,_______, and _______.

In older patients there may be uncontrolled proliferation of IgM-producing plasma cells with infiltrations of the _____ tract.

A

autoimmune hemolytic anemia, thrombocytopenia, and neutropenia

gastrointestinal

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36
Q

DiGeorge Syndrome (Thymic Hypoplasia)

T-cell deficiency that results from failure of ___________________

Due to __q__ —————

A

development of the third and fourth pharyngeal pouches.

22q11; microdeletion

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37
Q

DiGeorge Syndrome

Thus, individuals with this syndrome have a variable loss of T cell–mediated immunity (resulting from ___________), tetany (resulting from lack of the _______ ), and congenital defects of the __________. In addition, the appearance of the _________ may be abnormal

A

hypoplasia or lack of the thymus

parathyroids

heart and great vessels

mouth, ears, and facies

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38
Q

DiGeorge syndrome is a familial disorder.

T/F

A

F

not

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39
Q

DiGeorge syndrome

It results from the ____ of a gene that maps to chromosome ___

Absence of _______ immunity is caused by low numbers of T lymphocytes in the blood and lymphoid tissues and poor defense against certain _____ and ______ infections.

A

deletion; 22q11

cell-mediated

fungal and viral

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40
Q

In Digeorge syndrome

The T-cell zones of lymphoid organs—_______ areas of the lymph nodes and the ____________ of the spleen—are depleted.

Ig levels may be __________, depending on the _________________

A

paracortical; periarteriolar sheaths

normal or reduced; severity of the T-cell deficiency.

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41
Q

Severe Combined Immunodeficiency (SCID)

a constellation of genetically distinct syndromes, all having in common defects in ____________________________ immune responses

A

both humoral and cell-mediated

42
Q

Often, the SCID defect resides in the ___-cell compartment, with a secondary impairment of ______ immunity

A

T

humoral

43
Q

SCID

ETIOLOGY: Mainly 3-
________ (__) defect
____________ deficiency
__________ deficiency

A

Cytokine receptor(IL2)

Adenosine deaminase

MHC class II

44
Q

SCID

The most common form, accounting for 50% to 60% of cases, is ______

The remaining cases of SCID are inherited as ___________

A

X-linked

autosomal recessive.

45
Q

SCID

The genetic defect in the X-linked form is a mutation in the ________ subunit of cytokine receptors

The most common cause of autosomal recessive SCID is a ____________________

A

common γ-chain (γc)

deficiency of the enzyme adenosine deaminase (ADA)

46
Q

The histologic findings in SCID depend on the underlying defect.

In the two most common forms (ADA deficiency and γc mutation), the thymus is ____ and _______

A

small

devoid of lymphoid cells.

47
Q

In SCID caused by ADA deficiency, ___________ can be found, whereas in X-linked SCID the thymus contains lobules of ___________ cells resembling ___________

In either case other lymphoid tissues are hypoplastic as well, with marked depletion of ___-cell areas and in some cases both __-cell and __-cell zones.

A

remnants of Hassall’s corpuscles

undifferentiated epithelial ; fetal thymus.

T

T; B

48
Q

TREATMENT of SCID

•______ baby (_____)
•____transplant (HSC- B & T cells)

•Currently, ________ transplantation is the mainstay of treatment

A

Bubble; sterile isolation

Stem cell

bone marrow

49
Q

_________ is the first human disease in which gene therapy has been successful

A

X-linked SCID

50
Q

Wiskott-Aldrich Syndrome

an X-linked recessive disease characterized by _______,_____,_______, and ______
a marked vulnerability to ______, ending in early death.

A

thrombocytopenia, petechial haemorrhage, eczema, and skin rashes

recurrent infection

51
Q

The Wiskott-Aldrich syndrome is caused by mutations in the gene encoding ________, which is located at Xp_____.

This protein belongs to a family of proteins that are believed to link ________, such as antigen receptors, to ______ elements

A

Wiskott- Aldrich syndrome protein (WASP)

11.23

membrane receptors

cytoskeletal

52
Q

In WAS

The thymus is morphologically Abnormal from the start

T/F

A

F

Normal at least early in the course of the disease,

53
Q

In WAS

Patients do not ___________, and the __________ is poor.

A

make antibodies to polysaccharide antigens

response to protein antigens

54
Q

In WAS

IgM- ___
IgG- ______
IgE- _____
IgA- ____

A

low

normal

high

high

55
Q

In WAS, Patients are also prone to developing Hodgkin B-cell lymphomas
T/F

A

F

non- Hodgkin B-cell lymphomas

56
Q

Genetic Deficiencies of the Complement System

A deficiency of ____ is the most common of all.

A

C2

57
Q

Genetic Deficiencies of the Complement System

In c2 deficiency, there is high susceptibility to infections

A

F

there is little or no increase in susceptibility to infections,

58
Q

Genetic Deficiencies of the Complement System

In C2 deficiency, the dominant manifestation is an increased incidence of an _________ disease

Why??

A

SLE-like autoimmune

presumably, the alternative complement pathway is adequate for the control of most infections

59
Q

Complement system deficiency

Deficiency of components of the alternative pathway (______ and _____ ) is rare.

A

properdin and factor D

60
Q

Complement system deficiency

Deficiency of components of the alternative pathway
It is associated with recurrent pyogenic infections
The ___ component of complement is required for both the classical and alternative pathways
a deficiency of this protein results in _____________

A

C3; susceptibility to serious and recurrent pyogenic infections.

61
Q

Complement system deficiency

C3 deficiency

There is ___eased incidence of immune complex–mediated glomerulonephritis; in the absence of complement

A

incr

62
Q

The terminal components of complement C5, 6, 7, 8, and 9 are required for the assembly of the membrane attack complex involved in the lysis of organisms.
With a deficiency of these late-acting
components, there is increased susceptibility to recurrent __________
infections;

A

neisserial (gonococcal and meningococcal)

63
Q

Neisseria bacteria have (thin or thick ?) cell walls and are especially susceptible to the _________

A

Thin

lytic actions of complement

64
Q

A deficiency of C1 inhibitor gives rise to
__________

A

hereditary angioedema

65
Q

hereditary angioedema

This autosomal (recessive or dominant?) disorder is (more or less?) common than complement deficiency states.

A

Dominant; more

66
Q

The C1 inhibitor is a protease inhibitor whose target enzymes are ____ and ___ of the complement cascade, ______ of the coagulation pathway, and the _____ system

A

C1r and C1s

factor XII; kallikrein

67
Q

hereditary angioedema

These patients have episodes of edema affecting skin ( _______ ) and mucosal surfaces such as the _____ and the _________
This may result in life-threatening asphyxia or nausea, vomiting, and diarrhea after minor trauma or emotional stress.

A

peri-orbital

larynx

gastrointestinal tract.

68
Q

Acute attacks of hereditary angioedema can be treated with ________ prepared from ______

A

C1 inhibitor concentrates

human plasma.

69
Q

Deficiency of _________ is the cause of paroxysmal nocturnal hemoglobinuria.

A

other complement-regulatory proteins

70
Q

paroxysmal nocturnal hemoglobinuria.

In this disease there are mutations in enzymes required for _______ linkages, which are essential for the assembly of _______ and _______, both of which regulate complement.

A

glycophosphatidyl inositol

decay-accelerating factor and CD59

71
Q

Uncontrolled complement activation on the surface of red cells is believed to be the basis of _______ .

A

hemolysis

72
Q

Mutations in the complement regulatory protein factor ___ underlie about 10% of cases of a renal disease called _______________ syndrome, which is characterized by _______ in the kidneys

A

H

hemolytic uremic

microvascular thrombosis

73
Q

Presentation of Digeorge syndrome

CATCH-22:

A

Cardiac defects
Abnormal facies
Thymic hypoplasia
Cleft palate,
Hypocalcemia

74
Q

In SCID

Recurrent viral, bacterial, fungal, and protozoal infections e.g ___,____,_____,____,_____ and a whole host of _____.

A

Candida albicans, P. jiroveci, Pseudomonas, cytomegalovirus, varicella,

bacteria

75
Q

FINDINGS in SCID

•____________ (TRECs)  Part of newborn screening for SCID

•Absence of _____ (CXR), _______ (lymph node biopsy), and T cells (flow cytometry)

A

T-cell receptor excision circles

thymic shadow; germinal centers

76
Q

PRESENTATION of Wiskott-Aldrich

WAITER:

A

Wiskott-Aldrich:
Immunodeficiency
Thrombocytopenia,
Eczema,
Recurrent (pyogenic) infections

77
Q

The three major routes of transmission are:
_____ transmission:
______ transmission:
________ transmission

A

Sexual

Parenteral

Perinatal

78
Q

The probability of HIV-1 transmission is highest for

•_______(>___),

•followed by _____ transmission (about __)

•intermediate for _____ (about ___), and

•lowest for ______ transmission (about ____).

A

blood transfusion ; 0.95

mother to- child ; 0.1

needle-sharing; 0.01

woman-to-man sexual ; 0.001

79
Q

____ transmission: It is the main route of infection in more than ___% of cases of HIV.

A

Sexual

75

80
Q

Viral transmission can occur in two ways:

–__________ , and

— By ___________

A

Direct inoculation of virus or infected cells into the blood vessels at the site of breach caused by trauma

uptake into the mucosal dendritic cells (DCs).

81
Q

Parenteral transmission: Three groups of individuals are at risk._________

 ________

 ____________

A

Intravenous drug abusers

Hemophiliacs

Transfusion of blood or blood components

82
Q

HIV

Because of recently infected individual may be antibody- ______ ( ______ ), there is a small risk of acquiring AIDS through transfusion of blood.

A

negative; seronegative

83
Q

Organs from HIV-infected donors can also transmit AIDS.

T/F

A

T

84
Q

Perinatal transmission

 Transmission of infection can occur by three routes:–_____
–______
–_______

A

In utero

Perinatal spread

After birth

85
Q

Major mode of transmission of AIDS in children is by ????

A

Perinatal transmission

86
Q

HIV occurs in two genetically different but related main forms, _____ and ___

A

HIV-1 and HIV-2.

87
Q

HIV-1 is most common in the ____,____,______

HIV-2 is common in _______ and ____

A

United States, Europe, and Central Africa,

West Africa and India.

88
Q

HIV 1 is oncogenic

T/F

A

T

89
Q

Viral core of HIV

It contains
1. Major capsid protein ___
2. Nucleocapsid protein ____
3. Two identical copies of single stranded RNA genome.4. Three viral enzymes: 1)____, 2) _______ and 3) ______.

A

p24
p7/p9.

protease
reverse transcriptase
integrase

90
Q

______ and the antibodies against it are used for the diagnosis of HIV infection in enzyme-linked immunosorbent assay (ELISA).

A

Major capsid protein

91
Q

Lipid envelope of HIV

The virus contains a lipoprotein envelope, which consist of lipid derived from the host cell and two viral glycoproteins. These glycoproteins are: 1)______ which project as a knob-like spikes on the surface and 2)_____ anchoring transmembrane pedicle. These glycoproteins are essential for HIV infection of cells.

A

gp120

gp41

92
Q

HIV Genome

It contains two main groups of genes and their products act as antigens.

  1. Standard genes: HIV-1 RNA genome contains three standard retroviral genes, which are typical of retroviruses. These include:___,____, and _____ genes. Initially, the protein products of the gag and pol genes are translated into large precursor proteins and are later cleaved by the viral enzyme _____ to form mature proteins.
  2. Accessory genes: HIV contains accessory genes: e.g.__,___ vif, nef, and vpr, and vpu. They regulate the synthesis and assembly of infectious viral particles and the pathogenicity of the virus.
A

gag, pol, and env; protease

tat, rev,

93
Q

HIV-1 enters cells through interaction with the ____ receptor and a chemokine co-receptor (____ or ___).

The virus infects _____ cells and ____ expressing these receptors.

A

CD4; CXCR4 or CCR5

CD4-positive T; macrophages

94
Q

HIV-1 can also infect dendritic cells.

T/F

A

T

95
Q

HIV-1 can be assigned to one of three classes based on its ability to use the two co-receptors.

•Class __ :previously called ______ or __-tropic viruses.

•Class __ - previously called ______ or __-tropic viruses.

•Class _____ or ___ viruses.

A

R5: nonsyncytia - inducing (NSI) ; M

X4; syncytiainducing (SI); T

R5X4; dual

96
Q

CD4-positive T cells in lymphoid tissues can express only CCR5 receptors

T/F

A

F

both CCR5 and CXCR4, and are the main target for replication in vivo.

97
Q

Most individuals have the (R5 or X4?) virus at the time of diagnosis, whereas the presence of the (R5 or X4?) and __ virus is associated with progression to AIDS.

A

R5

X4; dual

98
Q

Early acute phase of HIV:

IT depends on blood
CD4+ T-cell count.
This divides the patients into three categories with counts being:

•________ cells/μL
•________ cells/μL
•__________ /μL = full blown AIDS

A

greater than 500

between 200 to 500

less than 200 cells

99
Q

People with healthy immune system usually have _____ CD4 cells/uL of blood

A

> 950

100
Q

______: Detects antibodies against viral proteins AND It is the most sensitive and best screening test for the diagnosis of AIDS.

________: Most specific or the confirmatory test for HIV.

A

ELISA

Western blot

101
Q

List 4 aids defining neoplasms

A

Anal carcinoma
cervical cancer
Kaposi sarcoma
Non Hodgkin Bcell lymphoma

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