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Pathology > Hypersensitivity, Autoimmunity and Immunodeficiency Part 4 > Flashcards

Hypersensitivity, Autoimmunity and Immunodeficiency Part 4 Flashcards

1
Q

what are histocompatability molecules

A
  • located on chromosome 6

- induce and regulate immune response

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2
Q

what do histocompatability molecules bind to

A

peptide of foreign proteins for presentation to appropriate T cells known as MHC restriction

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3
Q

what are human leukocyte antigen complex

A

MHC

highly polymorphic

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4
Q

where are class I MHC molecules HLA-A, HLA-B and HLA-C loci found

A
  • found on all nucleated cells

- trans-membrane alpha glycoprotein chain with a beta2- microglobulin

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5
Q

class I MHC molecules react with what

A

antibodies and CD8+ T lymphocytes

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6
Q

Class I MHC molecules are effective against what

A

virus infected cells and are principal agents involved in transplant rejection

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7
Q

how do you identify Class I MHC molecules

A

standard serologic techniques used for identification

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8
Q

how are HLA-D identified

A

only by mixed lymphocyte reactions, and sub-regions

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9
Q

how are HLA-DP, HLA-DQ, HLA-DR indentified

A

by either standard serologic techniques or mixed lymphocyte reactions

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10
Q

where are Class II Antigens HLA Associations found

A

on immune competent cells, including macrophages, dendritic cells, Langerhans cells, B cells and some T cells

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11
Q

trans-membrane alpha chain and beta chain react with what

A

CD4+ T lymphocytes

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12
Q

what is the function of trans-membane alpha chain and beta chain

A

effective against exogenous antigens processed by antigen presenting cells

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13
Q

what are class III proteins

A

complememnt components (C2, C3 and Bf)

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14
Q

what do class III protein genes encdoe for

A

TNF

LT or TNF-B

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15
Q

***what diseases are associated with HLA types

A
  • 90% of cases of Ankylosing spondylitis HLA-B27
  • primary hemochromatosis HLA-A3
  • 21- hydroxylase deficiency HLA-BW47
  • Rheumatoid arthritis HLA-DR4
  • insulin dependent diabetes mellitus HLA-DR2/DR3
  • systemic lupus erythematosus HLA-DR2/DR3
  • Uveitis HLA-B27
  • Reiter syndrome (urethritis, conjunctivitis and arthritis) HLA-B27
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16
Q

what is amyloidosis

A

-is a generic term for a variety of proteinaceous materials deposited in tissue interstitum in a spectrum of clinical disorders

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17
Q

what do amyloids look like in an H and E stain

A

is an amorphous, eosinophilic hyalin extracellular substance

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18
Q

what happens as amyloidosis progress

A

get adjacent pressure atrophy of adjacent cells

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19
Q

what is amyloidosis composed of

A

nonbranching fibril proteins 7.5 to 10nm in width by EM

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20
Q

x-ray crystallography and infrared spectroscopy of amyloidosis show a characteristic of

A

crossed Beta pleated sheet conformation

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21
Q

what are minor components of all amyloid deposits (5%)

A

-a nonfibrillar pentagonal glycoproteins known as the P component and proteoglycans

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22
Q

what are the most 3 common amyloidosis

A
  • AL (amyloid light chain) derived from plasma cells and contains immunoglobulin light chains
  • AA (amyloid-assocaited) is a unique non-immunoglobulin protein made in the liver
  • ABeta amyloid found in the cerebral lesion of Alzheimer disease
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23
Q

AL protein is made of what

A

complete immunoglobulin light chains, the NH2-terminal fragments of light chains or both

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24
Q

AL protein deposition is associated with what

A

some form of monoclonal B-cell proliferation as AL is produced by immunoglobulin secreting cells

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25
Q

AA amyloid fibril is composed of what

A

a protein of 8.5-kD molecular mass (76 aa resides) that does not have homology to immunoglobulins

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26
Q

when are AA proteins deposited

A

during chronic inflammatory states

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27
Q

AA amyloid fibrils are derived from what

A

a 12-kD serum precursor synthesized in the liver known as serum amyloid-associated protein (SAA)

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28
Q

what is transthyretin (TTR)

A

is a normal serum protein which binds and transports thyroxin and retinol

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29
Q

a mutatant TTR and its fragments are found deposited in a group of genetic disorders called

A

familial amyloid polyneuropathies

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30
Q

how does amyloid transthyretin (ATTR) differ from its normal counterpart

A

differs by 1 a.a

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31
Q

where is TTR found

A

in hearts of the elderly (senile systemic amyloidosis)

32
Q

Beta2- microglobulin (class I MHC antigen) has been identified as what

A

the amyloid fibril subunit (AM2m) in amyloidosis in the synovium, joints, and tendon sheaths of 60% to 80% of patients on long term hemodialysis and are structurally similar to the normal protein

33
Q

can the B2-microglobulin be filtered through the cuprophane dialysis membrane

A

no

34
Q

what is the beta-amyloid protein

A

is a 4-kD peptide that constitutes the core of the cerebral Alzheimer plaques as well as the amyloid deposited in cerebral blood vessel walls of Alzheimer patients

35
Q

ABeta protein is derived from what

A

-a larger transmembrane glycoprotein known as amyloid precursor protein (APP)

36
Q

what are amyloid deposits derived from

A

diverse precursors like hormones such as procalcitonin and keratin

37
Q

what are the classifications of amyloidosis

A
  • systemic or generalized involves several organ systems
  • localized where only one organ, such as the heart, are involved
  • primary amyloidosis is associated with immunocyte dyscrasias
  • secondary amyloidosis is a complication of chronic inflammatory or tissue destructive process
  • hereditary or familial is a separate, heterogenous group with distinctive patterns of organ involvement
38
Q

what are primary amyloidosis

A

systemic AL type

39
Q

what is multiple myeloma associated from

A
  • is an example of primary amyloidosis

- the Bence Jones proteins lambda and klight chains are produces as well as the M (myeloma) protein

40
Q

6% to 15% of meyloma patients develop what

A

amyloidosis so other factors (amyloidogenic potential) plays a role

41
Q

majority of patients with AL do not have what

A

overt B cell dyscrasia

42
Q

what are reactive systemic amyloidosis

A
  • systemic AA types

- secondary amyloidosis

43
Q

what is a common feature of reactive systemic amyloidosis

A

protracted cell injury

44
Q

what are common causes of reactive systemic amyloidosis

A

pre-antibiotic treatment for:
TB
Brochiectasis
Chronic osteomyelitis

45
Q

what is reactive systemic amyloidosis

A

a common cause of post antibiotic

46
Q

what are automimmune states of reactive systemic amyloidosis

A
  • Rheumatoid arthritis seen in 3% of pts
  • ankylosing spondylitis
  • inflammatory bowel disease
  • chronic skin infection as with “skin pippin”
  • non-immunocyte derived tumors such as renal cel carinoma and Hodgkin disease
47
Q

what is Heredofamilial Amyloidosis

A
  • rare and in limited geographic areas

- autosomal recessive familial Mediterranean fever

48
Q

what is Mediterranean fever

A
  • fever with inflammation of serosal surfaces in Armenians, Sephardic Jews and Arabs
  • systemic and AA
  • gene has been cloned and produces pyrin
49
Q

autosomal dominant familial disorders are characterized by what

A

deposition of amyloid in the peripheral and autonomic nerves, amyloidotic polyneuropahy found in Portugal, Japan Sweden and the US due to mutant tranthyretins (ATTR)

50
Q

what are localized amyloidosis

A

-nodular masses found in lung, larynx, skin urinary bladder, tongue and around the eye

51
Q

what do the nodules of localized amyloidosis look like

A

nodules rimmed by infiltrates of lymphocytes and plasma cells and the amyloid is AL

52
Q

what are endocrine amyloid

A

-are microscopic deposits found in medullary carincoma of thyroid, islet tumours of panreases, phenochromocytomas and undifferentiated cancer of the stomach, islets of Langerhans in NIDDM

53
Q

amyloid derived from polypeptide hormones or from

A

unique proteins such as islet amyloid polypeptide (IAPP)

54
Q

what are senile systemic amyloidosis

A

-are systemic deposits of amyloid in patients in their 70s or 80s, but usually found in the heart with restrictive cardiomyopathy and arrhythmias senile cardiac amyloidosis

55
Q

senile systemic amyloidosis are composed of what

A

normal transthyretin molecules

56
Q

4% of black population in the US are carriers for the allele for what

A

a mutant form of transthyretin leading to cardiac amyloidosis

57
Q

what is the pathogenesis of reactive systemic amylodosis

A
  • results from long standing tissue injury and inflammation which leads to formation of elevated SAA levels by liver cells under influence of IL-6 and IL-1
  • have enzyme defect resulting in incomplete breakdown of SAA by monocyte-derived enzymes, leading to the AA molecule formation
58
Q

in immunocyte dyscrasias the course of the precursor protein is derived

A

in vitro by proteolysis of the immunoglobulin light chains

59
Q

what is the pathogenesis of immunocyte dyscrasias

A

-defective proteolytic degradation has been evoked

60
Q

secondary amyloid due to what

A

chronic inflammation found in kidneys, liver, spleen, lymph nodes, adrenals and thryoid as well as other tissues

61
Q

where are immunocyte-associated amyloidosis found

A

heart, GI tract, respiratory tract, peripheral nerves, skin and tongue
-kidneys, liver and spleen

62
Q

where do Familial Mediterranean fever show deposits

A

in kidneys, blood vessels, spleen, respiratory tract and sometimes the liver

63
Q

iodine and surlfuric acid will lead to what color staining of amyloid

A

-mahogany brown staining of amyloid deposits on organ when deposits are too small to see grossly

64
Q

with increased deposition of amyloid what will happen to the organ

A

will become enlarged with a gray color and a way firm consistency

65
Q

histologically amyloid beigns between what

A

cells increasing to produce pressure atrophy of cells

66
Q

what are the locations of immunocyte-assocaited form

A

-perivascular and vascular localization

67
Q

what color do amyloids appear when stained with congo red

A
  • pink or red color under polarized light
  • the Congo red strained amyloid shows an apple green birefrigence shared by all forms of amyloid due to the crossed Beta-pleated configurations of the fibrils
68
Q

what can confirm the presence of amorphous nonoriented thin fibrils

A

EM

69
Q

what are initial symptoms of amyloidosis

A

weakness, fatigue, weight loss are initial symptoms

70
Q

what are other symptoms of amyloidosis

A

-renal disease with nephrotic syndrome and RF, hepatosplenomegaly, cardiac abnormalities with restrictive cardiomyopathy or arrhythmias
40% of patients with AL died of cardiac disease

71
Q

biopsy and congo red staining of kidney, rectum or gingiva, or abdominal fat aspirates 75% of patients with generalized amyloidosis will be

A

positive

72
Q

what tests should be performed in AL

A

-serum and urinary protein electrophoresis and immunoelectrophoresis should be performed

73
Q

what will bone marrow aspirate show in AL patients

A

plasmacytosis

74
Q

the prognosis of AA depends on what

A

on control of underlying condition

75
Q

Myeloma-associated amyloidosis has poor prognosis but may respond to treatment of what

A

myeloma

Pathology (18 decks)

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