Diseases of the Immune System IV Flashcards

1
Q

cellular rejection

A

T cell response to transplant

CD8 and CD4

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2
Q

direct pathway of cellular rejection

A

T cells recognize antigens on donor MHC on APCs in graft

CD4 and CD8 response

**acute rejection

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3
Q

indirect pathway of cellular rejection

A

T cells recognize antigens of donor on own APCs

CD8 response not generated
-bc these CTLs recognize graft antigens presented by hosts APCs

**chronic rejection

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4
Q

humoral rejection

A

B cell response to transplant

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5
Q

hyperacute humoral rejection

A

preformed antidonor antibodies present in recipient

-ex/ multiparous women against paternal antigens from fetus

or blood transfusions

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6
Q

acute humoral rejection

A

exposure to antigens of donor graft stimulate antibody production

often attack vessels first - vasculitis

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7
Q

hyperacute rejection

A

within minutes or hours

in kidney - glomeruli have thrombi occlusion and fibrinoid necrosis

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8
Q

acute rejection

A

within days

in kidneys - vasculitis (humoral) and interstitial mononuclear infiltrate (cellular)

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9
Q

acute cellular rejection

A

CD4 and CD8

kidney - mononuclear cells in capillaries, glomeruli, tubules

endothelitis

respond well to immunosupressive therapy (cyclosporine)

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10
Q

acute humoral rejection

A

kidney - mainly damage to vessels
-proliferation of smooth muscles

deposition of C4d (from classical complement pathway)

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11
Q

chronic rejection

A

kidney - progressive renal failure with rise in creatinine over period of 4-6 months

dense obliterative intimal fibrosis in cortical arteries

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12
Q

increasing graft survival

A

unless identical twins - immunosuppressive therapy necessary

cyclosporine 
azathioprine
steroids
monoclonal anti-T-cell antibodies
rapamycin
mycophenolate mofetil

also, attempt to prevent costimulatory signals
-interrupt B7 CD28 interaction

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13
Q

cyclosporine

A

blocks NFAT

-no IL-2 production

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14
Q

transplantation of hematopoietic cells

A

from bone marrow

problems:
graft-versus host disease
immunodeficiency

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15
Q

graft-versus host disease

A

immune competent cells transplanted to immune crippled recipient
-transferred cells recognize host antigens

often with bone marrow transplant

depleting T cells in donor bone marrow can help to eliminate disease

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16
Q

acute GVH

A

within days or weeks
-involve immune system, skin epithelia, liver, intestines

rash, jaundice, bloody diarrhe

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17
Q

chronic GVH

A

cutaneous injury
fibrosis of dermis (may resemble scleroderma)

liver problems - jaundice
esophageal strictures

18
Q

graft-versus-leukemia effect

A

T cells in bone marrow transplant control leukemic cells

19
Q

primary immunodeficiency

A

genetic

most manifest in infancy

  • 6 months to 2 years
  • with recurrent infection
20
Q

secondary immunodeficiency

A

due to infection, cancer, malnutrition, therapy, etc.

21
Q

X-linked agammaglobulinemia

A

failure of B cell precursors to develop to mature B cells

heavy change rearranged first
-induce light chain rearrangement

mutation in Btk - no light chain rearrangement

seen mostly in males - around 6 months of age
-infections - haemophilis influenza, streptococcus pneumoniae, traphylococcus auresu

also susceptible to viral infections

22
Q

Btk

A

mutated in X-linked agammaglobulinemia

-no light chain formation in B cells

23
Q

clinical features of X-linked agammaglobulinemia

A

B cells decreased
-serum antibodies are all decreased

-pre-B cells in normal numbers

underdeveloped germinal centers

absent plasma cells

T cell responses normal

24
Q

Tx of x-linked agammaglobulinemia

A

immunoglobulin replacement therapy

-allows most to reach adulthood

25
common variable immunodeficiency
hypogammaglobulinemia -sometimes only IgG normal B cells in blood - with no plasma cells **contrast with X-linked agammaglobulinemia
26
clinical features of common variable immunodeficiency
childhood or adolescence hyperplastic B-cell areas of lymphoid tissues (no negative feedback by antibodies) recurrent sinopulmonary pyogenic infection recurrent herpesvirus infection
27
isolated IgA deficiency
low levels of serum and secretory IgA mucosal defense weakened -infection of respiratory, GI, GU tracts if transfused with normal IgA - can develop anaphylactic reaction to IgA
28
hyper IgM syndrome
high IgM no IgM, IgA, IgE defect in ability of helper T cell to activate B cells - no class switching - mutation in CD40 hemolytic anemia thrombocytopenia neutropenia recurrent pyogenic infections
29
class switching
CD40 on B cells with CD40L on activated helper T cells
30
Digeorge syndrome
T cell deficiency failure development of 3rd and 4th pharyngeal pouches - no thymus (no T cells), parathyroid (tetany) - defects in heart and great vessels
31
22q11 deletion syndrome
leads to Digeorge syndrome
32
severe combined immunodeficiency
SCID defects in humoral and cell-mediated immunity infants with thrush, diaper rash, failure to thrive recurrent infections - candida albicans, P. jiroveci, pseudomonas Tx - bone marrow transplant necessary
33
X-linked SCID
more common in boys - mutation in cytokine receptor (gamma-c) - defect in T cell development - defect in B cells - no helper T cells also deficiency of NK cells
34
autosomal recessive SCID
deficiency of adenosine deaminase (ADA)
35
hassall corpuscles
seen in thymus of SCID with ADA deficiency
36
lobules of undifferentiated epithelial cells resembling fetal thymus
seen in thymus of SCID with gamma-c deficiency (X-linked)
37
Tx of SCID
bone marrow transplant also gene therapy - retroviral gamma-c gene introduction
38
wiskott-aldrich syndrome
immunodeficiency with thrombocytopenia and eczema X-linked recessive recurrent infections progressive decrease of T cells IgM low, IgG normal, IgE and IgA elevated mutation in WASP Tx bone marrow transplant
39
genetic deficiency in complement
most common - C2 -SLE -like autoimmune disease C3 - susceptible to infection
40
neisseria infection
have thin cell walls | -recurrent infections with deficiency of C5,6,7,8,9
41
hereditary angioedema
deficiency of C1 inhibitor
42
parenteral
intravenous