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IHO Week 5 > Immune Diseases #3 > Flashcards

Immune Diseases #3 Flashcards

1
Q

Autograft

A

self to self (ie skin)

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2
Q

Isograft

A

syngeneic, between identical twins

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3
Q

allograft

A

between genetically different individuals of same species

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4
Q

xenograft

A

between two species (ie porcine heart valve to human)

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5
Q

Major barrier to transplant success

A

rejection

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6
Q

what kinds of immunity involved in rejection of transplant

A

can involve both cell-mediated and antibody-mediated immunity

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7
Q

State the two groups of antigens that are most important in determining the likelihood of transplant rejection.

A

ABO and HLA

Key HLA loci involved in transplant rejection include HLA-A, HLA-B, HLA-C (minor importance), and HLA-DR.

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8
Q

T cell-mediated graft rejection is called

A

cellular rejection

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9
Q

Cellular rejection involves

A

destruction of donated graft cells by recipient CD8+ cytotoxic T lymphocytes and delayed hypersensitivity reactions triggered by activated recipient CD4+ T helper lymphocytes

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10
Q

Major antigenic differences between donor and recipient that results in rejection of transplants

A

differences in the highly pleomorphic HLA alleles

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11
Q

Two pathways for recipients T cells to recognize donor alloantigens

A

direct and indirect

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12
Q

Direct pathway

A

Donor class I and class II MHC antigens on antigen-presenting cells in the graft are recognized by host CD8+ cytotoxic T cells and CD4+ helper T cells, respectively. CD4+ cells proliferate and produce cytokines (e.g., IFN-γ), which induce tissue damage by a local delayed hypersensitivity reaction. CD8+ T cells responding to graft antigens differentiate into CTLs that kill graft cells.

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13
Q

Indirect pathway

A

In the indirect pathway graft antigens are picked up, processed, and displayed by host APCs and activate CD4+ T cells, which damage the graft by a local delayed hypersensitivity reaction and stimulate B lymphocytes to produce antibodies.

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14
Q

Antibodies produced against alloantigens in the graft are part of ____ rejection

A

humoral rejection

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15
Q

Important preformed anti-donor antibodies include:

A

Antibodies to ABO blood group antigens (naturally occurring)

Preformed anti-HLA antibodies (pregnancy, previous transfusion, previous transplant)

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16
Q

if preformed antibodies are present, a ________ reaction is possible

A

hyperacute rejection

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17
Q

How can antibodies to HLA antigens cause injury following transplantation?

A

several mechanisms, including complement-dependant cytotoxicity, inflammation, and antibody-dependant cell-mediated cytotoxicity. Antibody-mediated acute rejection is usually manifested in the vasculature, resulting in rejection vasculitis

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18
Q

key immunological factors affecting graft survival

A

ABO compatibility and close matching of HLA loci, along with an absence of preformed anti-HLA antibodies

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19
Q

Pre-transplant testing may include

A

ABO compatibility
HLA typing of donor and recipient
Detection of preformed anti-HLA antibodies
Lymphocyte cross-match (detect anti-HLA abs in recipients vs donor)

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20
Q

Hyperacute rejection d/t

A

ABO incompatibility and preformed anti-HLA abs in recipient

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21
Q

Hyperacute rejection steps

A

preformed ab bind endothelial antigens, activate complement, and result in vessel thrombi and ischemic necrosis (type II antibody-mediated hypersensitivity reaction).

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22
Q

hyperacute rejection when

A

minutes to hours following transplant

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23
Q

acute rejection can result from

A

from T cell-mediated hypersensitivity reactions (host CD4+ T-cells release cytokines, activating host macrophages, and CD8+ T-cells) or from antibody-mediated hypersensitivity reactions (host CD4+ T-cells release cytokines which promote B-cells to differentiate into plasma cells that produce anti-HLA antibodies that bind to endothelial antigens).

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24
Q

acute rejection when

A

days to weeks

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25
Q

chronic rejection

A

occurs over months and years and is often secondary to vascular injury, as a result of both cell-mediated and antibody-mediated hypersensitivity reactions.

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26
Q

Chances of graft survival are improved by

A

ABO and HLA matching, with absence of preformed anti-HLA antibodies
Use of immunosuppressive agent

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27
Q

Long term immunosuppressive therapy potential complications

A

increased susceptibility for opportunistic infections and malignancies

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28
Q

Autologous HCT

A

(auto-HCT) uses hematopoietic progenitor cells derived from the individual with the disorder.

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29
Q

Allogeneic HCT

A

allo-HCT) uses hematopoietic progenitor cells collected from someone other than the individual with the disorder.

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30
Q

underlying pathogenesis GVHD

A

the immunologically competent donor T cells recognize the recipient’s HLA antigens as foreign and react against them. Because the recipient (host) is immunocompromised (typically from myeloablative therapy), the host is incapable of mounting a reaction against the grafted lymphocytes, thus allowing the graft lymphocytes to attack the host.

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31
Q

autologous HCT and GVHD

A

Patients undergoing autologous HCT do not get GVHD

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32
Q

GVHD seen in what

A

allogeneic HCT, also liver transplants or transfusion of blood or platelets to immunocompromised host

33
Q

Acute GVHD

A

occurring in the first 100 days following allogeneic hematopietic cell transplantation. The mechanisms of injury involves direct cytotoxicity by CD8+ T cells as well as cellular injury caused by cytokines released from activated CD4+ T cells.

34
Q

Acute GVHD in what systems and symptoms

A

the skin, liver, and GI tract epithelium are most affected. Patients may experience severe dermatitis, destruction of small bile ducts with jaundice, and GI tract mucosal ulceration with bloody diarrhea.

35
Q

chronic GVHD

A

more than 100 days after allogeneic HCT, may follow acute GVHD or occur insidiously.

36
Q

chronic GVHD systems and symptoms

A

The skin may exhibit loss of skin appendages with dermal fibrosis. Chronic liver disease may result in cholestatic jaundice. The GI tract may exhibit fibrous strictures, along with malabsorption and chronic diarrhea. The lungs may show obliterative bronchiolitis.

37
Q

Explain why patients undergoing HCT are immunodeficient.

A

of prior treatment, myeloablative therapy in preparation for the graft, and delay in the repopulation (reconstitution) of the recipient’s immune system

38
Q

Common long-term toxicities of allogeneic HCT

A

●Chronic graft-versus-host disease (GVHD)
●Infections
●Treatment-related myelodysplasia/secondary leukemia
●Secondary solid tumors
●Cardiac disease
●Pulmonary toxicity

39
Q

Autologous HCT survivor long term toxicities

A 
●Treatment-related myelodysplasia/secondary leukemia 
●Secondary solid tumors 
●Cardiac disease 
●Pulmonary toxicity
●Infection
40
Q

group of disorders characterized by the deposition of amyloid in the extracellular space of tissues and organs, resulting in tissue and organ dysfunction

A

amyloidosis

41
Q

group of pathologic proteins that share similar physical properties

A

amyloid

42
Q

H&E Amyloid

A

On H&E staining, amyloid appears as an amorphous, eosinophilic, hyaline, extracellular substance that can result in pressure atrophy of adjacent cells.

43
Q

Amyloid Congo Red

A

It appears red with the Congo red stain and on polarization, the red stained amyloid exhibits a green birefringence.

44
Q

Amyloid structure

A

It consists of linear, non-branching fibrils in a characteristic cross-beta-pleated sheet configuration. This physical configuration is seen regardless of the chemical composition and results in characteristic staining properties.

45
Q

Is amyloidosis a single disease entity, or a group of diseases?

A

The various chemical types of amyloid are due to different pathogenic mechanisms. Thus amyloidosis is not a single disease, but a group of diseases having in common the deposition of similar appearing proteins.

46
Q

How does amyloid injure adjacent cells?

A

amorphous, eosinophilic, hyaline, extracellular substance that can result in pressure atrophy of adjacent cells.

47
Q

5 types of amyloid listed in class

A 
AL 
AA
beta-amyloid
Transthyretin
B2-microglobulin
48
Q

AL made up of

A

Amyloid Light Chain
either complete immunoglobulin light chains, the amino-terminal fragments of light chains, or both
usually lambda

49
Q

AL creation

A

is produced from free immunoglobulin light chain protein secreted by a monoclonal population of plasma cells (monoclonal lambda or kappa free light chain protein).

50
Q

AA made from

A

Amyloid-associated
derived by proteolysis of a larger precursor protein in the serum called SAA (serum amyloid-associated) protein that is synthesized in the liver and circulates in the serum associated with high density lipoprotein

51
Q

AA/SAA increased when

A

The production of SAA is increased in inflammatory conditions as part of the acute phase response; thus, this form of amyloidosis is associated with chronic inflammation.

52
Q

beta-amyloid protein made from

A

derived by proteolysis of a larger precursor protein called amyloid precursor protein.

53
Q

beta-amyloid found where

A

This form of amyloid is found in the cerebral plaques of Alzheimer disease as well as in the walls of the cerebral vessels of patients with Alzheimer disease.

54
Q

Transthyretin (TTR) amyloid from

A

normal serum protein that binds and transports thyroxine and retinol. Mutations of TTR can result in amyloid deposition, and these genetically determined disorders are referred to as heritable neuropathic and/or cardiomyopathic amyloidosis. TTR can also be deposited in the heart of aged individuals (as part of senile systemic amyloidosis) without mutation of TTR.

55
Q

B2-microglobulin from

A

this normal serum protein cannot be filtered through dialysis membranes and can accumulate in patients on long term dialysis (>20 years) and the condition is known as hemodialysis-associated amyloiodosis.

56
Q

prion diseases

A

In some cases of prion disease, amyloid protein can accumulate in the CNS as a localized form of amyloidosis.

57
Q

amyloidosis involving several organ systems

A

systemic

58
Q

amyloidosis involving a single organ (e.g. heart).

A

localized

59
Q

subclassifications systemic amyloidosis

A

primary

secondary

60
Q

primary amyloidosis

A

when associated with an clonal proliferative disorder of plasma cells producing light chain immunoglobulin

61
Q

secondary amyloidosis

A

when it occurs as a complication of an underlying chronic inflammatory process

62
Q

a group of heterogenous, heritable forms of amyloidosis with several distinctive patterns of organ involvement.

A

hereditary (familial) amyloidosis

63
Q

Typical distribution of amyloid deposition AL (primary)

A

Sites typically affected include heart, kidney, peripheral nerve, gastrointestinal tract, respiratory tract, but nearly any other organ can be involved. (Systemic)

64
Q

Underlying pathogenesis AL (primary)

A

Monoclonal proliferation of plasma cells (MM or underlying monoclonal plasma cell proliferation which produces an abnormal light chain protein, or ig secreting Bcell lymphomas)

65
Q

Reactive Systemic Amyloidosis AKA

A

as AA amyloidosis, secondary amyloidosis, second most common type of amyloidosis in the USA

66
Q

Reactive Systemic Amyloidosis is secondary to

A

chronic inflammatory condition, such as rheumatoid arthritis, other CT disordrs, IBD
Can also be seen associated with renal cell carcinoma and Hodgkin lymphoma

67
Q

Most common type of amyloidosis in developing world

A

AA amyloidosis is the most common cause of amyloidosis, due to chronic inflammation associated with infectious disease (e.g. TB, leprosy, osteomyelitis).

68
Q

Sites commonly affected with reactive, systemic, amyloidosis

A

Kidney, liver, spleen

69
Q

hemodialysis-associated amyloidosis deposition of what

A

to deposition of amyloid derived from beta-2 microglobulin, which accumulates in patients with end-stage renal disease who are being maintained for prolonged periods of time by dialysis

70
Q

where does Hemodialysis-associated amyloidosis deposit

A

osteoarticular structures

71
Q

Familial Mediterranean Fever

A

Autoinflammatory syndrome
Autosomal recessive
Mutation in gene that produces proteins that regulate inflammatory reactions (AA protein)

72
Q

Familial amyloidotic neuropathies

A

disorders associated with mutant forms of TTR

73
Q

Age-related (senile) Systemic amyloidosis due to

A

amyloid deposition associated with normal (wild-type) TTR protein.

74
Q

Senile systemic amyloidosis normally involves

A

heart –> restrictive cardiomyopathy and arrhythmias

renal rarely

75
Q

Localized amyloidosis

A

Amyloid deposits limited to a single organ or tissue without involvement of other tissues or sites.

76
Q

clinical symptoms of systemic amyloidosis

A

waxy skin and easy bruising, enlarged muscles (e.g. tongue, deltoids), symptoms and signs of heart failure, cardiac conduction abnormalities, hepatomegaly, renal dysfunction (heavy proteinuria or the nephrotic syndrome), peripheral and/or autonomic neuropathy, and impaired coagulation (acquired factor X deficiency due to this coagulation factor binding to amyloid)

77
Q

Diagnosis of amyloidosis

A

tissue biopsy with staining for amyloid. Biopsy can be directed at a suspected clinically involved organ (e.g. kidney, nerve) or a clinically uninvolved site if systemic disease is suspected (fat pad aspiration, rectal biopsy)

78
Q

Treatment amyloidosis

A

Varies with the cause of amyloid production.
Treat underlying infectious or inflammatory disorder in AA amyloidosis
Treat monoclonal plasma cell proliferation in AL amyloidosis
Either alter mode of dialysis or consider renal transplantation in patients with dialysis-related amyloidosis

IHO Week 5 (3 decks)

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