Immunodeficiency - lecture 1 Flashcards
leucocyte adhesion deficiencies
disorders of phagocyte migration - failure to express adhesion molecules on vascular endothelium results in inability of phagocytes to exit blood stream.
which primary phagocyte deficiency lacks pus formation and neutrophil infiltration
leucocyte adhesion deficiencies
which primary phagocyte deficiency has high neutrophil count in the peripheral blood
leucocyte adhesion deficiencies
chronic granulomatous disease
failure of oxidative killing
results in inability to clear organisms - excessive inflammation and granulomatous formation
which primary phagocyte deficiency shows abnormal results in NBT test
chronic granulomatous disease
defects in cytokines and cytokines receptors
e.g. IFN - gamma and IL-12 or their receptors results in failure of intracellular killing
which primary phagocyte deficiency makes individuals susceptible to mycobacterial infections
defects in cytokines and cytokines receptors
3 types of primary phagocyte deficiency
chronic granulomatous disease
leucocyte adhesion deficiency
defects in cytokines and cytokines receptors
complement pathway deficiency causes individuals to present with recurrent infection with ________ bacteria
encapsulated
deficiency of classical complement pathway (C1, C4, C2) is associated with
autoimmune disease
individuals with mannose binding lectin deficiency have increase incidence of _____ infection if subjected to _______
bacterial
additional cause of immunocompromised e.g. premature birth or chemotherapy
primary deficiencies of adaptive immune system - 3 examples
digeorge syndrome
bare lymphocyte syndrome
autoimmune lymphoproliferative syndrome
digeorge syndrome caused by
failure of development of pharyngeal pouch, usually cause by deletion of 22q11
digeorge syndrome characterised by
low numbers of mature T cells
bare lymphocyte syndrome characterised by
absent expression of HLA molecules in thymus
bare lymphocyte syndrome - failure of HLA class I to develop causes
failure of CD8+ T cells to develop
bare lymphocyte syndrome - failure of HLA class II to develop causes
failure of CD4+ T cells to develop
autoimmune lymphoproliferative syndrome is
failure of apoptosis of lymphocytes
combined T and B lymphocyte deficiencies
severe combined immune deficiency (SCID) is caused by failure of T and B cells to mature
treatment for SCID
bone marrow transplant
primary antibody deficiencies - 3 examples
selective IgA deficiency
common variable immune deficiency
specific antibody deficiency / functional IgG deficiency
most common primary antibody deficiency
selective IgA deficiency
selective IgA deficiency - sometimes compensatory increase in ___ levels
IgG levels
CVID characterised by
low serum IgG levels
failure to make antibody response to exogenous pathogens
