Immunodeficiency Flashcards
What causes immunodeficiency?
Defects in one or more components of immune system leading to immunodeficiency disease
Primary immunodeficiency
Genetic defects
Secondary immunodeficiency
Caused by infection, immunosuppressive drugs, or malnutrition.
Hypogammaglobulinemias
Humoral, Primary. Occurs at birth that is normal. Delayed B-lymphocyte development.
Bruton’s agammaglobulinemia
Humoral, Primary (x-linked). Deficiency of mature B cells due to deletion of B cell tyrosine kinase. T cell normal. Infection begins after maternal IgG declines.
Hyper-IgM syndrome
Humoral, Primary (x-linked). B lymphocytes cannot undergo isotype switching. Cannot switch to heavy chain do to defects.
IgA deficiency
Humoral, Primary. Most common immunodeficiency. Lots of respiratory infections, mucosal infections, ciliac disease.
Common variable immunodeficiency (CVID)
Humoral, Primary. Most common. Mature B cells are present but plasma cell are absent. Lots of malignant tumors.
DiGeorge syndrome
Cellular,Primary. Defects in T lymphocytes maturation, absent or greatly deficient. Normal B cells. Malformed thymus, viral infection increase. Can transplant new thymus.
Severe, combined immunodeficiency (SCID).
Primary (inherited). Both humoral and cell-mediated. Bubble boy. Reduced B and T cells. Bone marrow transplant for treatment.
Chronic granulomatous disease (CGD)
Primary. Phagocygtes cannot produce H2O2 and radical O2. Defect in NADPH oxidase. Inject with IFN-y to activated macrophages for controlling bacterial and fungal infections.
What is the first opportunistic infection with HIV patients?
Oral candidiasis
How does HIV enter CD4+ T helper cells?
HIV gp120 binds to CD4 molecule.
What destroys CD4+ T helper cells in HIV?
CD8 T cytotoxic cells. CD4 T helper cell present to CD8 T cytotoxic cell on MHC1.
When is aids status achieved?
around 1/10 of T-helper cells than normal levels