MODULE IV Flashcards

1
Q

what are the examples of inappropriate and pathologic responses?

A

immunodeficiency (primary, secondary)
hypersensitivity
gammopathy
autoimmune disease or immunopathology

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2
Q

what are the types of immunodeficiencies?

A
primary or genetic
secondary or acquired
cells and systems involved 
-phagocyte (nonspecific dysfunction)
-complement (non/specific dysfunction)
-humoral (specific dysfunction)
-cell mediated (specific dysfunction)
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3
Q

what are the warning signs of immune deficiency?

A
2 or more ep of pneumonia
4 of more episodes of otitis
recurrent stomatitis
recurrent abcess
1 episode of severe systemic infection
recurrent intestinal infection
severe asthma
adverse affect to BCG
clinical phenotype suggestive
family history
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4
Q

effect of B cell deficiencies?

A

bacterial infections

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5
Q

effect of T cell deficiencies?

A

viral and other intracellular microbial infections

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6
Q

innate immune deficiencies?

variable; pyogenic bacterial infections

A

chronic granulomatous disease

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7
Q

name this immune system deficiency

absent or deficient expression of B2 integrins causing defective leukocyte adhesion dependent functions

A

leukocyte adhesion deficiency 1

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8
Q

name this immune system deficiency

absent or deficient expression of leukocyte ligands for endothelial E and P selectins, causing failure of leukocyte migration into tissues

A

leukocyte adhesion deficiency 2

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9
Q

name this immune system deficiency

defect in complement cascade activation

A

complment C3 deficiency

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10
Q

name this immune system deficiency

deficient activation of classical pathway of complement leading to failure to clear immune complexes and development of lupus like disease

A

complement of C2, C4 deficiency

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11
Q

name this immune system deficiency

defective lysosome function

A

Chediak-Higashi syndrome

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12
Q

what type of defect is this?

recurrent non superficial pyogenic infections and recurrent ENT and airway infections

A

phagocytic cell defects

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13
Q

name this phagocytic cell defect

this is a killing defect where there is a lack of NADPH oxidase (therefore decrease ROS)

A

chronic granulomatous disease (CGD)

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14
Q

name this phagocytic cell defect

this is a chemotaxis defect with high IgE, staph infections, lung disease, eczema, rare

A

hyper IgE syndrome (Job syndrome)

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15
Q

name this phagocytic cell defect

chemotaxis defect with defects in neutrophil chemotaxis, adhesion, and activation issues

A

leukocyte adhesion defect (LAD)

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16
Q

name this phagocytic cell defect

chemotaxis defect in which there is a failure to thrive

A

schwachman-diamond syndrome

17
Q

name this phagocytic cell defect

chemotaxis defect with ROS

A

chediak higashi syndrome

18
Q

what are the immunologic and nonimmunologic characteristics of Hyper-IgE syndrome?

A

immunologic characteristics

  • newborn rash
  • boils
  • eczema
  • elevated IgE
  • eosinophilia

nonimmunologic characteristics

  • characteristic face
  • retained primary teeth
  • hyperextensibility
19
Q

defect in leukocyte adhesion and migration responsible for neutrophilia or neutropenia

A

Leukocyte adhesion deficiency (LAD)

20
Q
mutation in SBDS gene
diarrhea, failure to thrive, malabsorption til 4 mo
eczema, recurrent infections 
neutrophil could < 1000, pancytopenia 
risk of AML
A

Shwachman Diamond Syndrome

21
Q

autosomal recessive disorder characterized by partial occulocutaneous albinism, grayish white hair, giant granules in many cells, neutropenia and recurrent infections

chromosomal mutation 1q43 affecting Lyst gene

A

chediak higashi syndrome

22
Q

a type of cytotoxic lymphocyte that is not class restricted?

these play a major role in rejection of tumors and cells infected by viruses

the cells kill by releasing small cytoplasmic granules of proteins causing apoptosis

A

natural killer cells

23
Q

affect of classical NK cell deficiency?

A

Qty in peripheral circulation

24
Q

affect of functional NK cell deficiency?

A

function deficiency

25
why are NK cells important?
involved in viral and cancerous cell removal
26
this is a stem cell immune deficiency that lacks both B and T cells in peripheral circulation lacks T and B cells in lymphoid tissues genetic defect in synthesis of adenosine deaminase (ADA) or purine nucleoside phosphorylase (PNP)
severe combined immunodeficiency disease (SCID)
27
how is SCID treated?
HSCT transplant enzyme replacement therapy bone marrow transplant
28
a stem cell immune deficiency rare AR neurodegenerative Louis bar syndrome none immune symptoms, early childhood gait disruption, movement and eye movement immunodeficiencies with T cells. IgA, IgG2 no treatment, 20-25 year life expectancy
ataxia telangiectasia
29
a stem cell immune deficiency x-linked genetic disorder thrombocytopenia and progressive T cell functional reactivity loss eczema and repeated bacterial infections treatment: bone marrow transplant and antimicrobials normal IgG, reduced IgM
wiskott-aldrich syndrome
30
name this B cell immunodeficiency rare absence of all Ig isotypes, normal T cell function lack B cell tyrosine kinase
X linked infantile agammaglobulinemia (Brutons's)
31
name this B cell immunodeficiency lack of IgG to IgM synthesis, normal IgA no IgM from mother B cells are normal and in peripheral circulation treatment: gamma globulin and no immunizations
transient infantile hypogammaglobulinemia
32
name this B cell immunodeficiency ``` onset around 15-35 years B cells become unresponsive to Th signals, IgA class switching is most affected ``` increased risk of lymphomas in females treatment: IV immunoglobulin
common variable immunodeficiency (CVID)
33
name this B cell immunodeficiency IgA- most common with sinopulmonary infections IgG2/4- increased susceptibility to pyogenic bacterial infections IgM- recurrent infections with encapsulated bacterias and septicemias Hyper IgM- Inability to carry out class switching so accompanied by IgG/IgA deficiency treatmentL IVIG except for IgA deficiency
dysgammaglobulinemias-selective immunoglobulin deficiency
34
name this T cell deficiency? lack of development of thymus and parathyroid during fetal development thyme dysplasia or aplasia few or no T cells in peripheral circulation or in T cell areas of regional lymphoid tissues no production of IgG
DiGeorge syndrome
35
name this T cell immunodeficiencies? thyme dysplasia affects T cell maturation abnormal IgGa dn reduced CMI with some T cells present
Nezelof's syndrome
36
name this T cell immunodeficiencies? defect in T cell responsiveness to Candida. normal Ig response IL-2/IFN-g/IL-10
chronic mucocutaneous candiasis
37
what are examples of secondary immune deficiencies?
``` HIV infection diabetes immune suppresion malnutrition, vitamin and mineral deficiency autoimmune disorders ```