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Pathology > Immuno4 0519FA > Flashcards

Immuno4 0519FA Flashcards

1
Q

Bruton’s agammaglobulinemia

A

B cell disorder.
X-linked recessive.
more in Boys.

defect in BTK tyrosine kinase gene blocks pro-B cells from forming pre-B cells.

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2
Q

presentation of Bruton’s agammaglobulinemia

A

recurrent BACTERIAL infxs after 6 mos (decreased maternal IgG) due to opsonization defect

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3
Q

labs in Bruton’s agammaglobulinemia

A

normal pro-B.
decreased B maturation.
decreased #B cells.
decreased all classes of Ig.

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4
Q

hyper-IgM syndrome

A

B cell disorder.

defective CD40L on helper T cells = inability to class switch.

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5
Q

presentation of hyper-IgM syndrome

A

severe pyogenic infxs early in life

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6
Q

labs in hyper-IgM syndrome

A

INCREASED IgM.

greatly decreased IgG, A, E.

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7
Q

selective Ig deficiency

A

B cell disorder.

defect in isotype switching = deficiency in specific class of Igs.

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8
Q

presentation of selective Ig deficiency

A

sinus and lung infx.
milk allergies, diarrhea.
ANAPHYLAXIS on exposure to blood products with IgA.

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9
Q

labs in selective Ig deficiency

A

IgA deficiency most common.
failure to mature into plasma cells.
decreased secretory IgA.

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10
Q

common variable immunodeficiency (CVID)

A

B cell disorder.

defect in B cell maturation due to various causes.

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11
Q

presentation of CVID

A

can be acquired age 20-30.

increased risk of:
AUTOIMMUNE disease.
lymphoma.
sinopulmonary infx.

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12
Q

labs in CVID

A

NORMAL number of B cells.

decreased plasma cells and Ig.

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13
Q

thymic aplasia (DiGeorge syndrome)

A

T cell disorder.

22q11 deletion.
failure to develop 3rd and 4th pharyngeal pouches.

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14
Q

presentation of thymic aplasia (DiGeorge syndrome)

A

tetany (hypocalcemia).
recurrent viral/fungal infxs.
congenital heart and great vessel defects.

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15
Q

labs in thymic aplasia (DiGeorge syndrome)

A

failure of thymus and parathyroids to develop = decreased T cells, PTH, and serum calcium.

ABSENT THYMIC SHADOW on CXR.

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16
Q

IL-12 receptor deficiency

A

T cell disorder.

decreased Th1 response.

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17
Q

presentation of IL-12 receptor deficiency

A

disseminated mycobacterial infxs

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18
Q

labs in IL-12 receptor deficiency

A

decreased IFN-gamma

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19
Q

hyper-IgE syndrome (Job’s syndrome)

A

T cell disorder.

Th cells fail to produce IFN-g = inability of neutrophils to respond to chemotactic stimuli.

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20
Q

presentation of hyper-IgE syndrome (Job’s syndrome)

A 
FATED:
coarse Facies.
cold, noninflamed staph Abscesses.
retained primary Teeth.
increased IgE.
Derm problems (eczema).
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21
Q

chronic mucocutaneous candidiasis

A

T cell disorder.

T cell dysfunction leading to Candida albicans infx of skin and mucous membs.

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22
Q

severe combined immunodeficiency (SCID)

A

B and T cell disorder.

various types:

  1. defective IL-2 receptor: most common type. X-linked.
  2. adenosine deaminase deficiency.
  3. failure to synthesize MHC II Ags.
23
Q

SCID presentation

A

recurrent bacterial, viral, fungal, AND protozoal infxs.

ABSENT THYMIC SHADOW.
absent germinal centers (LN).
absent B cells in peripheral smear.

24
Q

SCID labs

A
  1. decrease IL-2 receptor = decrease T cell activation.

2. increased adenosine with ADA deficiency = toxic to B and T cells (decreased dNTP, decreased DNA synth)

25
Q

SCID TX

A

BM transplant (no allograft rejection)

26
Q

ataxia-telangiectasia

A

B and T cell disorder.

auto recessive defect in ATM gene that codes for DNA repair enzymes.

27
Q

presentation of ataxia-telangiectasia

A

TRIAD:

  1. cerebellar defects (ataxia).
  2. spider angiomas/oculocutaneous lesions (telangiectasias).
  3. IgA deficiency.
  • DNA is hypersensitive to ionizing radiation.
  • increased sinopulmonary infxs and malignancy.
28
Q

Wiskott-Aldrich syndrome

A

B and T cell disorder.

X-linked recessive defect.
PROGRESSIVE DELETION of B and T cells.

29
Q

presentation of Wiskott-Aldrich syndrome

A

TRIAD (TIE):

  1. Thrombocytopenic purpura.
  2. Infxs (encapsulated orgs).
  3. Eczema.
30
Q

labs in Wiskott-Aldrich syndrome

A

increased IgE, IgA.

decreased IgM.

31
Q

leukocyte adhesion deficiency type I

A

phagocyte dysfunction.

defect in LFA-1 integrin (CD18) protein on phagocytes.

32
Q

presentation of leukocyte adhesion deficiency type I

A

recurrent bacterial infxs (skin).
absent pus formation.
poor wound healing.
delayed separation of umbilical cord.

NEUTROPHILIA.

33
Q

Chediak-Higashi syndrome

A

phagocyte dysfunction.

auto recessive defect in lysosomal trafficking regulator gene (LYST) = microtubule dysfunction in phagosome-lysosome fusion.

34
Q

presentation of Chediak-Higashi syndrome

A

recurrent pyogenic infx by staphylococcus, streptococcus.
partial albinism.
peripheral neuropathy.

35
Q

chronic granulomatous disease (CGD)

A

phagocyte dysfunction.

lack of NADPH oxidase = decreased ROS (superoxide) and absent resp burst in neutrophils

36
Q

presentation of CGD

A

increased susceptibility to CATALASE-POSITIVE organisms (S.aureus, E.coli, Aspergillus)

37
Q

landmark lab in CGD

A

negative Nitroblue tetrazolium dye reduction test

38
Q

autograft

A

from self

39
Q

syngeneic graft

A

from identical twin or clone

40
Q

allograft

A

from nonidentical indiv of same species

41
Q

xenograft

A

from different species

42
Q

hyperacute transplant rejection

A

within minutes.

Ab-mediated (type II) due to PREFORMED anti-donor Abs in transplant recipient.

43
Q

features of hyperacute transplant rejection

A

occlusion of graft vessels (acute thrombosis) causing ischemia and necrosis

44
Q

acute transplant rejection

A

weeks later.

cell-mediated due to cytotoxic T cells reacting against foreign MHCs.

45
Q

acute transplant rejection is reversible with…?

A

immunosuppressants (cyclosporine, OKT3).

try to prevent with calcineurin inhibitors and corticosteroids.

46
Q

features of acute transplant rejection

A

vasculitis of graft vessels with dense interstitial LYMPHOCYTIC infiltrate.

decreased organ function.

47
Q

chronic transplant rejection

A

months to years.
nonself MHC I is perceived by cytotoxic T cells as self MHC I presenting a nonself Ag.
may involve B cell sensitization.

NOT reversible.

48
Q

features of chronic transplant rejection

A

T cell and Ab-mediated vascular damage: OBLITERATIVE vascular fibrosis.

fibrosis of graft tissue and bld vessels.

49
Q

graft vs. host disease (GVHD)

A

onset varies.
grafted immunocompetent T cells proliferate in the irradiated immunocompromised host and reject cells with “foreign” proteins, thus causing severe organ dysfunction.

50
Q

features of GVHD

A

maculopapular rash.
jaundice.
hepatosplenomegaly.
diarrhea.

51
Q

GVHD occurs most often with what types of transplants?

A

bone marrow and liver

organs rich in lymphocytes

52
Q

GVHD is potentially beneficial in what kind of transplant?

A

bone marrow

53
Q

prevention of GVHD

A

HLA matching.

anti-thymocyte immune globulin to remove donor T cells.

Pathology (10 decks)

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