Immune Deficiencies Flashcards
Primary immunodeficiency is usually of ……origin
Genetic
Secondary immunodeficiency is the result of complications from……
1)
2)
3)
1) aging
2) infections
3) SE of immunosupressive therapies (i.e., chemotherapy)
Phagocyte (myeloid) defect leads to increase….
bacterial infections
Chronic Granulomatous disease
cannot produce superoxide radical
Myeloperoxidase deficency
cannot kill intreacellular bacteria
Leukocyte Adhesion Deficiency I and II
defects in integrin (I) or sleectin (II) decrease activation and migration of cells to sites of infection
Lymphocyte defects lead to ……
broad based immuen deficiencies
Why do lymphocyte defects lead to broad based immuen deficiencies?
pathogens can mutate outcompete preformed PAMP and cannot be controleld
Define severe combined immunodeficiency
1) rare defect in lymphoid stem cell
2) defects in T cell development
3) Lack of T cell development results in lack of B cell development
CD40L is located on….
the CD4 T cell that is bound to CD40 receptor on immature b cell
Define Digeorge Syndrome
- thymic aplasia because of defect in 3rd and 4th pharyngeal pouches
- thymic stroma can’t efficently support T cell development and maturation
Robust antibody production boosting of ab responses and generation of B cell memory depends on
CD4 Th2 T cell
Defects in antibody oposinization causes…
Recurrent bacterial infections
Defects in ADCC causes…
Parasitic infections
IgA defect leads to….
respiratory, GI, GU infections
defects in T cells propsenstiy to produce different types leads to….
1) decreased propensity to produce ab
2) amount and type of ab made
Benefits of materanl ab….
partially protects fetus and neonate during the first few months of life
*can obscure defects present in the neonate’s immune system
Which Ig is passively transferred from mother to fetus?
IgG
*Define X-linked Agammaglobulinemia
- males, because only have one X chromosome
1) increased infections at 8-9 months
2) defect in signaling molecule (bruton’s tyrosine kinase)
3) no mature B cells relased from bone marrow
4) no GC in lymph nodes, spleen or tonsils, no plasma cells, no serum Ig
Defect in bruton’s tyrosine kinase is assoc with
X-linked Agammaglobulinemia
X-linked Agammaglobulinemia lacks 3 things
1) no mature B cells relased from bone marrow
2) no gc in lymph nodes, spleen, or tonsil
3) no plasma cells, NO SERUM IG
Hyper IgM syndrome is what linked?
70% x-linked
- can have some phenotype from other mutation
Hyper IgM Syndrome is associated with….infections
Recurrent bacterial infections
List 5 things about hyper IgM
1) 70% X linked: can have same phenotype from other mutations
2) recurrent bacterial infections
3) T cell defect prevents B cell maturation
4) only IgM in the serum
5) no gc in lymph nodes spleen or tonsil
Protein calorie malnutrition
1) braod spectrum immunodeficiency
2) secondary immunodefiency
Irradation and Chemotherapy
1) decrease cell-mediated immunity
2) leukopenia, thrombocytopenia, neutropenia
Cancer Metastases to bone
1) broad spectrum immunodefiency
Immunosupressive Drug
1) supress all of immune system
Functions of the spleen
1) removal of unwanted elements of the blood
2) major secondary organ of immune system (dc in the spleen trap antigens and present them to Tcells)
Source of hematopoietic cells in cases of severe anemia
Spleen
Major clinical manifestation of removal of spleen
1) increased susceptibility to disseminated infection w/ encapsulated bacteria
- penumococcus
- meningococcus
- Haemophilus Influenzae
