Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

primary immunodeficiencies are

A

inherited and may affect any part of the immune system

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

when are primary immunodeficiencies apparent

A

-early in life as conferred maternal immunity wanes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

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

A

plasma cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

XLA, Bruton’s Disease has normal —– responses

A

T-cell mediated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

how do you treat XLA, Bruton’s Disease

A

-treatment with transfusion of IV Ig from pooled serum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

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

A

autoimmune disease such as RA and dermatomyositis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

what is common variable immunodeficiency (CVID)

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

how does CVID differ from XLA

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

patients with CVID have a relative high incidence of what

A

autoimmune disorders and lymphoreticular neoplasias

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

what is the most common primary immunodeficiency disorder

A

isolated IgA Deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

isolated IgA deficiency is associated with what other disease

A

autoimmune diseases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

what levels are normal in patients with isolated IgA deficinecy

A

-have normal or supranormal levels of IgM and IgG subclasses

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
what happens to IgA in patients with isolated IgA deficiency
-have a block in terminal differentiation of IgA secreting B cells to plasma cells
26
what is X-linked hyper- IgM (HIGM)
-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
27
what is normal in HIGM
-have normal or supranormal levels of IgM, but lack the ability to produce IgG, IgA or IgE isotypes
28
the CD40L gene is located on what chromosome
x chromosome, 70% of HIGM clases are x-linked
29
what are other causes of HIGM
-defect in activation induced deaminase and enzyme involved in class switching
30
what is X-HIGM
-defect in cell mediate immunity as CD40-Cd40L interaction is critical for helper t cell mediated activation of macrophages in cell mediated immunity
31
male patients with X-HIGM present with what
recurrent pyogenic infections due to low levels of opsonizing IgG and have a variety of intracelular pathogens inducing Pneumocystis jirovenic (carinii)
32
DiGeorge Syndrome is a result from what
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
DiGeorge Syndrome has a lack of what
T cells in lymph nodes, spleen and peripheral blood
34
infants with DiGeorge Syndrome are extremely vulnerable to what
viral, fungal, and protozoal infections and intracellular bacteria
35
are B cells affected in DiGeorge Syndrome
B cells and serum immunoglobulins are unaffected
36
patients with DiGeorge syndrome may have parathyroid hypoplasia leading to
hypocalcemic tetany and other midline development abnormalities including the face and aortic arch
37
90% of DiGeorge Syndrome cases have a deletion in what chromosome
22q11
38
what is severe combined immunodeficiency (SCID)
-a group of conditions with leukocytopena, impaired cell-mediated immunity, low or absent antibody levels and undeveloped secondary lymphoid tissues
39
patients with SCID have an autosomal recessive
adenosine deaminase deficiency or purine nucleoside phosphorylase deficiency
40
what type of disease is SCID
X-linked or autosomal recessive traits where enzymes are not affected but due to an inability to recombine antigen receptor molecules
41
different forms os SCID may be correlated to points of what pathway
lymphomyeloid differentiation pathway
42
what type of disease is Wiskott-Aldrish Syndrome
X-linked
43
what are the symptoms of Wiskott-Aldrich Syndrome
-thrombocytopenia, eczema and susceptibility to recurrent infections leading to early death
44
how do you treat Wiskott-Aldrich Syndrome
-bone marrow transplantation
45
what is the mechanism of Wiskott-Aldrich Syndrome
- 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
patients with Wiskott-Aldich Syndrome are prone to developing
malignant lymphomas
47
genetically, how does Wiskott-Aldrich Syndrome occur
-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
what are genetic deficiencies of components of innate immunity
-hereditary deficiencies in complement components (C3 classical and alternative pathways) result in increased susceptibility to infection with pyogenic bacteria
49
deficiencies in --, -- and -- increase the risk of immune complex-mediated disease (SLE)
C1q C2 C4
50
deficiencies in C1q, C2, C4 impair the clearance of what
apoptotic cells or antigen-Ab complexes
51
deficiencies of C5-C8 of the classical pathway result in recurrent infections by
Neisseria gonococci and meningococci only
52
lack of regulatory protein C1 inhibitor allows increased
C1 activation increased vasoactive complement mediators and results in hereditary angioedema
53
there are congenital defects if phagocytes including defects of pahgocyte oxidase enzyme leading to
chronic granulomatous disease and defects in integrins and selectin ligands with resultant leukocyte adhesion deficiencies
54
primary immune deficiency disease are caused by what
mutations in genes involved in lymphocyte maturation or function in innate immunity leading to increased susceptibility to infections in early life
55
XLA failure of B-cell maturation
lack of antibodies; mutations in BTK gene
56
CVI
defects in Ab production, unknown cause
57
IgA deficiency
failure of IgA production, cause unknown
58
X-SCID failure
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
autosomal SCID
- 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
X-linked hyper IgM syndrome
failure to produce isotype-switched high affinity antibodies (IgG, IgA, IgE; mutation in gene encoding CD40L)
61
secondary immune deficiencies are ---- that primary immune disorder
more common
62
where are secondary immune deficiencies found
in malnutrition, infection, cancer, renal disease or sarcoidosis
63
what is the most common cause of secondary immune deficiencies
-therapy induced suppression of the bone marrow and of lymphocyte function
64
AIDs is a retroviral disease caused by
HIV
65
AIDs is characterized by what
infection and depletion of CD4+ | T cells are profound immunosuppression with opportunistic infections, secondary neoplasms, and neurologic manifestations

Pathology (18 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions