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20 Cards in this Set
- Front
- Back
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What is a primary immunodeficiency |
PIDDs are due to intrinsic deficits within the immune systems and are typically caused by inherited disorder
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What are the symptoms of PIDDs |
infections; frequent severe and unsual organisms
autoimmune disease
immune dysregulation leading to hematopoetic malignancy |
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What are the difference presentations in the different arms of the immune system |
B cell defs = recurrent pyogenic bacterial infections, get CA infections just at a faster more frewuent rater
T cell defs = viral and other intracellular microbial infections
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What are the effects of humoral deficiencies |
recurrent sinpulmonary infections
onset after 6 mos of age, due to passive transfer of mom to baby |
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What are some of the specific antibody deficiencies |
Agammglobulinemia, x-linked
Hyper IgM syyndrome
Common Variable Immunodeficienct CVID |
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What are the characterisitcs of X-linked agammaglobulinemia |
mutation in the Btk gene, arrests development of pre B cells
Serum IgG IgA and IgM are very low
severe infections with encapsulated organisms with chronic enterovirak |
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How do we determine the general population of T and B cells |
CD3 (T) and Cd19 (B) the population in flow cytometry with no Cd19 or Cd3 are NK cells |
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What are the characteristics of CVID |
common respiatory pathogens
variable disease and infections, onset may be in childhood or adulthood, increased risk for malignancy or autoimune
non-uniform disease inheritance
immune defect is abnromal serum Ig levels and functional Abs, some have T cell defects |
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What are the characterisitics of hyper-IgM syndrome |
due to the abscence of T cell CD40L or B cell CD40L preventing the interaction preventing the T cell from activating B cell to undergo class switching
X linked recessive or AR
Dx using Ab levels and check for B cells |
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How do we treat Ab disorders |
use Ig replacement therapy |
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What are the cellular immundeficient disorders |
T cells usually in the macrophage activation or cytotoxic killing
Th1 cell most susceptible to mycobacteria, BCG, salmonella on the IL-12 and IFN gamma axis |
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What are defects that affect the whole T cell compartment |
Combined immunodeficiency
bacteria, virus, fungi and protozoa |
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What is Wiskott Aldrich Syndrome |
x linked disorder
triad of eczema, thrombocytopenia with small defective platelets, and recurrent infections
present with bleeding before infections |
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What is DiGeorge syndrome |
microdeletion in chromosome 22q11.2
abrnormal development in 3 and 4 pharyngeal pouches
usually thymus issues, not always a complete deletion
T cell proliferation is usually normal |
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What are the treatments for cellular or combined immunodeficiencies |
stem cell transplantation
thymic transplantation for complete DiGeorge syndrome |
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What are the effects of phagocyte defects |
Most common infection organisms
bacteria pseudomonas slamonell
mycobacteria and fungi |
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What is Leukocyte Adhesion Deficiency 1 |
component of leukocyte adhesion molecules necessary for cell adhesion and migration
neutrophils cannot migrate out of the blood vessels into the areas of infection
can cause omphalitis or delayed umbilical cord separation |
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What is chediak higaski syndrom |
causes albinism
platelet dysfunction
abnormal Nk function and neutrophil killing
Due to disorder of lysosomal compartment, cytotoxic granules cant properly fuse with endosomes membrane instead they self fuse and get stuck |
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How do we evaulate neutrophils |
NBT or DHR assays |
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What are the effects of complement deficiency |
early complement deficiency C1-4 often resulting in autoimmune disease
late component deficiency C5-9 reucrrent neisseria infections |
