Ch 6: Diseases of the Immune System Flashcards

1
Q

what is innate (natural) immunity

A

intrinsic mechanisms that are poised to react immediately and are the first line of defense against microbes and damaged cells
include inflammation and antiviral defense

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2
Q

what is adaptive (aquired) immunity

A

mechanisms that are stimulated by exposure to microbes and other foreign substances

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3
Q

define immunity

A

your body’s reaction against cancers, tissue transplants, and self antigens

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4
Q

what are the 6 main cell types involved in innate immunity

A

epithelial
monocytes
neutrophils
plasma proteins
dendritic cells
innate lymphoid cells - natural killer cells

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5
Q

explain the inflammation portion of innate immunity

A

cytokines and complement proteins trigger vascular and cellular componenets of inflammation
leukocytes destroy microbes as well as ingest and eliminate damaged cells
helps to repair damaged tissues

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6
Q

explain the antiviral defense portion of innate immunity

A

type 1 interferons act on infected cells and activate enzymes that degrade viral nucleic acids and inhibit viral replication

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7
Q

what are the two main cell types of adaptive immunity

A

T lymphocytes and B lymphocytes

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8
Q

what are the three types of T lymphocytes

A

helper T
killer (cytotoxic) T
regulatory T

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9
Q

what do helper T lymphocytes do

A

stimulate B lymphocytes to make antibodies and activate other leukocytes to destroy microbes

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10
Q

what are the two primary lymphoid organs

A

thymus and bone marrow

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11
Q

what are the 3 types of secondary lymphoid organs

A

nodes
spleen
mucosal and cutaneous (tonsils and adenoids)

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12
Q

thymus (6-25yrs)

A

25g

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13
Q

thymus (26-35yrs)

A

20g

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14
Q

thymus (36-65yrs)

A

16 g

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15
Q

thymus (65+ yrs)

A

6g

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16
Q

what color is most lymphoid tissue under the microscope and why

A

very purple because lots of lymphocytes

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17
Q

spleen (16-20yrs)

A

150-200g (170g)

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18
Q

spleen (20-65yrs)

A

155g

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19
Q

spleen (80+yrs)

A

100g

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20
Q

what is a splenuculus

A

accessory spleen
ususally 1-2cm

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21
Q

where are peyer’s patches found

A

in the GI tract

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22
Q

what are the four main types of immunologic disorders

A

hypersensitivity
autoimmune
immunologic deficiency syndrome
amyloidosis

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23
Q

define atopy

A

tendency to develop immediate hypersensitivity reactions

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24
Q

what three things that hypersensitivity reactions can be caused by

A

exogenous and endogenous antigens
susceptible genes
imbalance between effector mechanisms of immune responses and control mechanisms that serve to normally limit response

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25
Q

what is immediate (type 1) hypersensitivity

A

rapid reaction occuring in minutes anfter antigen binds
IgE antibody is produced
vasoactive amines (histamine) and other mediators from mast cells are released
recruitment of inflammatory cells
leads to vascular dilation, edema, smooth muscle contractions, and inflammation
ex. anaphylaxis, allergies, and asthma

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26
Q

what is antibody-mediated (type 2) hypersensitivity and what are two examples

A

production of IgG and IgM which bind to antigen on target cell
cell is phagocytosed, inflammed, or its functions disturbed
ex. autoimmune hemolytic anemia and Goodpasture syndrome

27
Q

what is immune complex mediated (type 3) hypersensitivity - give three examples

A

deposition of antigen-antibody complexes
leads to fever, inflammation, tissue damage, lymph node enlargement, and necrotizing vasculitis (fibrinoid necrosis)
ex. system lupus, serum sickness, and glomerulonephritis

28
Q

what is cell-mediated (type 4) hypersensitivity and give four examples

A

cell-mediated hypersensitivity
activated T lymphocytes release cytokines which cause inflammation
destruction by cytotoxic T cells also can cause inflammation
ex. contact dermatitis, multiple sclerosis, type 1 diabetes, and TB

29
Q

what is autoimmune hemolytic anemia

A

an example of a type 2 hypersensitivity reaction
immune system mistakes red blood cells as unwanted substance
your body produces antibodies that destroy red blood cells which leads to anemia

30
Q

what is Goodpasture syndrome and what are the two things it presents with

A

an example of a type 2 hypersensitivity reaction
antibodies against collagen in the glomeruli and alveolar basement membrane
presents with: hematuria (blood in urine) and hemoptysis (coughing up of blood)

31
Q

what is anti-glomerular basement membrane antibody (ABMA)

A

antibody in Goodpasture Syndrome

32
Q

what is crescentic glomerulonephritis

A

seen in Goodpasture syndrome
crescent shape collection of collagen in bowman’s capsule of glomeruli of the kidneys

33
Q

what are the three phases of immune complex mediated (type 3) hypersensitivity

A

formation of immune complex
deposition of the immune complexes in various tissues
inflammatory reaction dispered through the body

34
Q

what is an Arthus reaction

A

an example of a type of immune complex mediated (type 3) hypersensitivity
localized area of tissue necrosis resulting from acute immune complex vasculitis

35
Q

what is an example of delayed-type hypersensitivity

A

type 4 hypersensitivity
tuberculin reaction from injection of purifed protein derivative made of a protein-containing antigen of the tubercle bacillus

36
Q

what percentage of the population has an autoimmune disease

A

5-10%

37
Q

why are women of childbearing age more likely to get an autoimmune disease

A

estrogen may reduce apoptosis of self-reactive B cells

38
Q

what is systemic lupus erythematosus (SLE) and what does it present with

A

a type of autoimmune disorder that affects 1 in 500
antigen-antibody complexes damage multiple tissues
get production of autoantibodies
half have renal involvement - diffuse lupus nephritis
can have libman-sacks endocarditis
presents with: malar butterfly rash, alopecia, synovitis, and discoid rash

39
Q

what is diffuse lupus nephritis

A

occurs in 50% of patients with systemic lupus erythematosus
glomeruli show proliferation of endothelial, mesangial, and epithelial cells that fill Bowmann’s space
shows wire loop on light microscopy

40
Q

what is Libman-Sacks (murantic or verrucous) endocarditis

A

a form of nonbacterial thrombotic endocarditis (NBTE)
sterile vegetations on cardiac valves due to endothelial injury
seen in those with systemic lupus erythematosus

41
Q

what is Sjogren Disease

A

chronic disease due to lymphocytic infiltration and fibrosis of the lacrimal and salivary glands
leads to: keratoconjunctivtis sicca (dry eyes) and xerostomia (dry moth)

42
Q

what is systemic sclerosis (scleroderma)

A

disease of excessive dense collagen deposition in the dermis
possible result of autoimmunity
widespready damage to small blood vessels
progressive interstital and perivascular fibrosis in skin and multiple organs such as the GI tract (EGJ specficially)
Raynaud’s disease is a symptom of this disease

43
Q

what is the Raynaud Phenomenom

A

numbness and tingling of the fingers and toes caused by episodic vasoconstriction of arteries and arterioles
characteristic of systemic sclerosis (scleroderma)

44
Q

what happens in hyperacute rejection of the kidney

A

immediate
cyanotic, mottled, and anuric kidney
arteries have fibrinoid necrosis in lumen
kidney eventually undergoes outright necrosis

45
Q

what is rejection

A

the process in which T lymphocytes and antibodies are produced against graft antigens react against and destroy tissue
three types: hyperacute, acute, and chronic

46
Q

what happens in acute rejection of the kidney

A

days or weeks
cytotoxic T-cells start destroying graft cells
helper T-cells release cytokines which produce inflammation that damages grade cells
shows up with wavy basement membranes in the tubules

47
Q

what happens in chronic rejection of the kidney

A

months or years
interstitial fibrosis and gradual narrowing of the graft blood vessels

48
Q

what are primary immunodeficiencies

A

inherited defects in the early innate immune response typically affect leukocyte functions or the complement system
all lead to increased vulnerability to infections

49
Q

what are the two main types of secondary immunodeficiencies (aquired)

A

HIV and amyloidosis

50
Q

what is DiGeorge Syndrome (Thymic Hypoplasia) and what is it caused by

A

a type of primary immunodeficiency
T-cell deficiency that results from failure of development of the thymus
usually caused by a small germline deletion that maps to chromsome 22q11

51
Q

what is Wiskott-Aldrich Syndrome (WASp protein)

A

a type of primary immunodeficiency
X-linked disease in the WAS protein
leads to increase susceptibility to infections and anemia

52
Q

what does WASp do

A

it’s a key actin cytoskeleton regulator that coordinates the assembly of actin filaments in response to cell signaling event

53
Q

what is selective IgA deficiency

A

most common type of primary immunodeficiency
impaired differentiation of naive B lymphocytes to IgA-producing plasma cells
leads to weakened mucosal defenses and infections in the respiratory, GI, and urogenital tracts

54
Q

what is acquired immunodeficiency syndrome (AIDS)

A

immunosupressions that leads to infections and cancers
second leading cause of dead in US men

55
Q

what are the three ways in which you can get HIV

A

sexual transmission (most common)
parenteral transmission (blood transfusions)
mother-to-infant (vaginal birth or ingestion of breast milk)

56
Q

what is human immunodeficiency virus (HIV)

A

human retrovirus from the lentivirus family
infects memory and activated T cells
loss of memory cell has ripple effects on every componenet of immune system

57
Q

what is candidiasis

A

most common fungal infection in patients with AIDS

58
Q

what is Kaposi sarcoma

A

most common neoplasm in patients with AIDS
proliferation of spindle-shaped cells with intervening blood filled channels

59
Q

90% of patients demonstrate some form of neurologic involvement (usually progressive encephalopathy) at autopsy when they have which disease

A

HIV

60
Q

what disease has the highest risk after a needlestick

A

Hepatitis C virus (HCV)

61
Q

what is amyloidosis

A

type of secondary immunodeficiency
inherited and inflammatory disorders in which extracellular deposits of fibrillar proteins (amyloid) cause tissue damage
amyloidosis condition caused by abnormal folding of proteins

62
Q

which stain tests for amyloidosis

A

congo red

63
Q

what is sago spleen

A

uniform enlargement of white pulp in spleen due to amyloid deposits
multiple tiny pearl like appearance of spleen