MODULE IV Flashcards
what are the examples of inappropriate and pathologic responses?
immunodeficiency (primary, secondary)
hypersensitivity
gammopathy
autoimmune disease or immunopathology
what are the types of immunodeficiencies?
primary or genetic secondary or acquired cells and systems involved -phagocyte (nonspecific dysfunction) -complement (non/specific dysfunction) -humoral (specific dysfunction) -cell mediated (specific dysfunction)
what are the warning signs of immune deficiency?
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
effect of B cell deficiencies?
bacterial infections
effect of T cell deficiencies?
viral and other intracellular microbial infections
innate immune deficiencies?
variable; pyogenic bacterial infections
chronic granulomatous disease
name this immune system deficiency
absent or deficient expression of B2 integrins causing defective leukocyte adhesion dependent functions
leukocyte adhesion deficiency 1
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
leukocyte adhesion deficiency 2
name this immune system deficiency
defect in complement cascade activation
complment C3 deficiency
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
complement of C2, C4 deficiency
name this immune system deficiency
defective lysosome function
Chediak-Higashi syndrome
what type of defect is this?
recurrent non superficial pyogenic infections and recurrent ENT and airway infections
phagocytic cell defects
name this phagocytic cell defect
this is a killing defect where there is a lack of NADPH oxidase (therefore decrease ROS)
chronic granulomatous disease (CGD)
name this phagocytic cell defect
this is a chemotaxis defect with high IgE, staph infections, lung disease, eczema, rare
hyper IgE syndrome (Job syndrome)
name this phagocytic cell defect
chemotaxis defect with defects in neutrophil chemotaxis, adhesion, and activation issues
leukocyte adhesion defect (LAD)
name this phagocytic cell defect
chemotaxis defect in which there is a failure to thrive
schwachman-diamond syndrome
name this phagocytic cell defect
chemotaxis defect with ROS
chediak higashi syndrome
what are the immunologic and nonimmunologic characteristics of Hyper-IgE syndrome?
immunologic characteristics
- newborn rash
- boils
- eczema
- elevated IgE
- eosinophilia
nonimmunologic characteristics
- characteristic face
- retained primary teeth
- hyperextensibility
defect in leukocyte adhesion and migration responsible for neutrophilia or neutropenia
Leukocyte adhesion deficiency (LAD)
mutation in SBDS gene diarrhea, failure to thrive, malabsorption til 4 mo eczema, recurrent infections neutrophil could < 1000, pancytopenia risk of AML
Shwachman Diamond Syndrome
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
chediak higashi syndrome
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
natural killer cells
affect of classical NK cell deficiency?
Qty in peripheral circulation
affect of functional NK cell deficiency?
function deficiency
why are NK cells important?
involved in viral and cancerous cell removal
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)
how is SCID treated?
HSCT transplant
enzyme replacement therapy
bone marrow transplant
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
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
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)
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
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)
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
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
name this T cell immunodeficiencies?
thyme dysplasia affects T cell maturation
abnormal IgGa dn reduced CMI with some T cells present
Nezelof’s syndrome
name this T cell immunodeficiencies?
defect in T cell responsiveness to Candida.
normal Ig response
IL-2/IFN-g/IL-10
chronic mucocutaneous candiasis
what are examples of secondary immune deficiencies?
HIV infection diabetes immune suppresion malnutrition, vitamin and mineral deficiency autoimmune disorders
