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Pathology > Hypersensitivity, Autoimmunity and Immunodeficiency Part 3 > Flashcards

Hypersensitivity, Autoimmunity and Immunodeficiency Part 3 Flashcards

1
Q

immunodeficiency disorders increase susceptibility to

A

infection due to failure of one or more divisions of the ummune system

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2
Q

primary immunodeficiencies are

A

inherited and may affect any part of the immune system

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3
Q

secondary (acquired) immunodeficiencies are a consequence of what

A

many pathogenic infections or other disease states which may directly attack the immune system or may subvert effective immune responses

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4
Q

primary immunodeficiencies are affected by what

A

-failure of lymphoctye development
-impaired granulocyte function
lack of macrophage receptors
-absence of particular complement complement components

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5
Q

when are primary immunodeficiencies apparent

A

-early in life as conferred maternal immunity wanes

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6
Q

what is X-linked agammaglobulinemia (XLA, Bruton’s Disease)

A

-patients have normal T cell function and cell-mediate immunity to viral infections but have very low immunoglobulin levels and cannot make Ab response

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7
Q

in XLA, Bruton’s Disease B cells fail to express what

A

a tyrosine kinase BTK, required for the maturation of pre-B cells to mature B cells

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8
Q

in XLA, Bruton’s DIsease when does B cell maturation stop

A
  • after initial heavy chain gene rearrangement due to the mutation in BTK associated with the pre-B cell receptor and involved in pre B-cell signal transduction
  • no Ig light chains are produced though heavy chains may be found in the cytoplasm
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9
Q

XLA, Bruton’s Disease is absent or markedly decreased numbers of what

A
  • B cells in circulation with depressed levels of all classes of immunoglobulins
  • Pre-B-cells in bone marrow are normal or reduced
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10
Q

in XLA, Bruton’s Disease what cells are underdeveloped

A

-underdeveloped, rudimentary germinal centers in lymph nodes, Peyer’s patches, appendix and tonsils

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11
Q

XLA, Bruton’s Disease has an absence of what cells

A

plasma cells

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12
Q

XLA, Bruton’s Disease has normal —– responses

A

T-cell mediated

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13
Q

when is XLA, Bruton’s Disease apparent

A

-at about 6 months of age with recurrent acute and chronic pharyngitis, sinusitis, ottitis media, bronchitis and pnaeumonia due to organisms typically cleared by antibody-mediated opsonization and phagocytosis

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14
Q

patients with XLA, Bruton’s Disease are susceptible to what

A
  • H. influenza, S. pneumonia or S. aureus

- Giardia lamblia due to lack of IgA

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15
Q

how do you treat XLA, Bruton’s Disease

A

-treatment with transfusion of IV Ig from pooled serum

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16
Q

20% of XLA, Bruton’s Disease may develop what

A

autoimmune disease such as RA and dermatomyositis

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17
Q

what is common variable immunodeficiency (CVID)

A

-a variety of conditions with no clear pattern of inheritance, which affect B cell differentiation

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18
Q

how does CVID differ from XLA

A

-sexes affected equally and symptoms occur in the 2nd or 3rd decade of life

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19
Q

what cells are abnormal in CVID

A

-B cells are present, but do not develop into plasma cells bc of lack of T cell help, T-cell suppressor activity, or intrinsic B-cell defects

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20
Q

patients with CVID have a relative high incidence of what

A

autoimmune disorders and lymphoreticular neoplasias

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21
Q

what is the most common primary immunodeficiency disorder

A

isolated IgA Deficiency

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22
Q

what is the function of IgA

A

is a major Ig in mucosal secretions therefore patients with isolated IgA deficiency have airway and GI defense problems

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23
Q

isolated IgA deficiency is associated with what other disease

A

autoimmune diseases

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24
Q

what levels are normal in patients with isolated IgA deficinecy

A

-have normal or supranormal levels of IgM and IgG subclasses

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25
Q

what happens to IgA in patients with isolated IgA deficiency

A

-have a block in terminal differentiation of IgA secreting B cells to plasma cells

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26
Q

what is X-linked hyper- IgM (HIGM)

A

-an immunodeficiency due to mutation in CD40L, the T cell ligand which binds to CD40 on B cells- an interaction required for class switching in secondary responses

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27
Q

what is normal in HIGM

A

-have normal or supranormal levels of IgM, but lack the ability to produce IgG, IgA or IgE isotypes

28
Q

the CD40L gene is located on what chromosome

A

x chromosome, 70% of HIGM clases are x-linked

29
Q

what are other causes of HIGM

A

-defect in activation induced deaminase and enzyme involved in class switching

30
Q

what is X-HIGM

A

-defect in cell mediate immunity as CD40-Cd40L interaction is critical for helper t cell mediated activation of macrophages in cell mediated immunity

31
Q

male patients with X-HIGM present with what

A

recurrent pyogenic infections due to low levels of opsonizing IgG and have a variety of intracelular pathogens inducing Pneumocystis jirovenic (carinii)

32
Q

DiGeorge Syndrome is a result from what

A

failed development of the 3rd and 4th pharyngeal pouches which leads to thymic hypoplasia with low #’s of functionally active T cells
-T cell number may rise to normal within 1-2 yrs of life thymus tissue transplant is effective

33
Q

DiGeorge Syndrome has a lack of what

A

T cells in lymph nodes, spleen and peripheral blood

34
Q

infants with DiGeorge Syndrome are extremely vulnerable to what

A

viral, fungal, and protozoal infections and intracellular bacteria

35
Q

are B cells affected in DiGeorge Syndrome

A

B cells and serum immunoglobulins are unaffected

36
Q

patients with DiGeorge syndrome may have parathyroid hypoplasia leading to

A

hypocalcemic tetany and other midline development abnormalities including the face and aortic arch

37
Q

90% of DiGeorge Syndrome cases have a deletion in what chromosome

A

22q11

38
Q

what is severe combined immunodeficiency (SCID)

A

-a group of conditions with leukocytopena, impaired cell-mediated immunity, low or absent antibody levels and undeveloped secondary lymphoid tissues

39
Q

patients with SCID have an autosomal recessive

A

adenosine deaminase deficiency or purine nucleoside phosphorylase deficiency

40
Q

what type of disease is SCID

A

X-linked or autosomal recessive traits where enzymes are not affected but due to an inability to recombine antigen receptor molecules

41
Q

different forms os SCID may be correlated to points of what pathway

A

lymphomyeloid differentiation pathway

42
Q

what type of disease is Wiskott-Aldrish Syndrome

A

X-linked

43
Q

what are the symptoms of Wiskott-Aldrich Syndrome

A

-thrombocytopenia, eczema and susceptibility to recurrent infections leading to early death

44
Q

how do you treat Wiskott-Aldrich Syndrome

A

-bone marrow transplantation

45
Q

what is the mechanism of Wiskott-Aldrich Syndrome

A
  • normal thymus with progressive age-related depletion of T cells and loss of cellular immunity
  • cannot synthesize Abs to polysaccharide antigens even though B cell response to these antigens do not require T cell help
46
Q

patients with Wiskott-Aldich Syndrome are prone to developing

A

malignant lymphomas

47
Q

genetically, how does Wiskott-Aldrich Syndrome occur

A

-the gene maps to the X chromosome and encodes the Wiskott-Aldrich protein that links several membrane receptors to the cytoskeleton leading to abnormal cell-cell adhesion and leukocyte migration

48
Q

what are genetic deficiencies of components of innate immunity

A

-hereditary deficiencies in complement components (C3 classical and alternative pathways) result in increased susceptibility to infection with pyogenic bacteria

49
Q

deficiencies in –, – and – increase the risk of immune complex-mediated disease (SLE)

A

C1q
C2
C4

50
Q

deficiencies in C1q, C2, C4 impair the clearance of what

A

apoptotic cells or antigen-Ab complexes

51
Q

deficiencies of C5-C8 of the classical pathway result in recurrent infections by

A

Neisseria gonococci and meningococci only

52
Q

lack of regulatory protein C1 inhibitor allows increased

A

C1 activation increased vasoactive complement mediators and results in hereditary angioedema

53
Q

there are congenital defects if phagocytes including defects of pahgocyte oxidase enzyme leading to

A

chronic granulomatous disease and defects in integrins and selectin ligands with resultant leukocyte adhesion deficiencies

54
Q

primary immune deficiency disease are caused by what

A

mutations in genes involved in lymphocyte maturation or function in innate immunity leading to increased susceptibility to infections in early life

55
Q

XLA failure of B-cell maturation

A

lack of antibodies; mutations in BTK gene

56
Q

CVI

A

defects in Ab production, unknown cause

57
Q

IgA deficiency

A

failure of IgA production, cause unknown

58
Q

X-SCID failure

A

T and B cell maturation mutation of the common V chain of cytokine receptor leading to failure of IL-7 signaling and defective lymphopoiesis

59
Q

autosomal SCID

A
  • failure of T-cell development, secondary defect in antibody responses
  • approx 50% of cases due to mutation of gene encoding ADA leading to accumulation of toxic metabolites
60
Q

X-linked hyper IgM syndrome

A

failure to produce isotype-switched high affinity antibodies (IgG, IgA, IgE; mutation in gene encoding CD40L)

61
Q

secondary immune deficiencies are —- that primary immune disorder

A

more common

62
Q

where are secondary immune deficiencies found

A

in malnutrition, infection, cancer, renal disease or sarcoidosis

63
Q

what is the most common cause of secondary immune deficiencies

A

-therapy induced suppression of the bone marrow and of lymphocyte function

64
Q

AIDs is a retroviral disease caused by

A

HIV

65
Q

AIDs is characterized by what

A

infection and depletion of CD4+

T cells are profound immunosuppression with opportunistic infections, secondary neoplasms, and neurologic manifestations

Pathology (18 decks)

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