Immunodeficiency Diseases Flashcards
What is primary immunodeficiency?
A genetic defect that results in an increased susceptibility to infection. Frequently manifested in infancy and childhood
What is secondary immunodeficiency?
A susceptibility to infection that developed as a consequence of malnutrition, disseminated cancer, treatment with immunosuppressive drugs, or infection of immune cells.
Any loss of function mutation that affects a _____ has negative consequences for survival.
Toll-Like Receptor
A defect in leukocyte maturation or activation will result in _____ immunodeficiency; whereas, a defect in effector mechanisms will result in ______ immunodeficiency.
Innate
Adaptive
Immunodeficiencies may affect one or more components of the immune system including _____ and _______ of the adaptive immune system.
T lymphocytes
B lymphocytes
Immunodeficiencies may affect one or more components of the immune system including _____ and _______ of the adaptive immune system.
T lymphocytes
B lymphocytes
What is the principal consequence of an immunodeficiency?
Increased susceptibility to infection
The types of ______ ______ are indicative of the type of immunodeficiency.
Recurring Infections
Deficient ______ immunity usually results in increased susceptibility to infection by pyogenic bacteria.
humoral
Boys with XLA have low/absent levels of _______ and display very small tonsils and lymph nodes.
B cells
Patients with hyper-IgM immunodeficiency have swollen lymph nodes due to a defect in _______ ligand on helper T cells that signals B cells to go through isotype switching.
CD40
*without isotype switching there is an elevated level of IgM and very low concentrations of IgG, IgA, and IgE
Deficient humoral immunity is usually treated via ________ and/or gamma-globulin therapy.
Prophylactic antibiotics
Deficient humoral immunity is usually treated via ________ and/or gamma-globulin therapy.
Prophylactic antibiotics
Deficient _______ immunity usually results in increased susceptibility to viruses and other intracellular pathogens.
Cell-mediated
What is the difference in susceptibility between humoral and cell-mediated immunity?
Humoral = pyogenic bacteria Cell-mediated = viruses and intracellular pathogens
True or False: Treatment for humoral immunodeficiency is routine, but there are few treatments for deficient T cell response (cell-mediated).
True
DiGeorge’s syndrome is not an immune specific disorder but rather a disease associated with the lack of _______ development.
Thymus
*B cell function is only impaired to the extent of needing helper T cells
X-linked _____ is the most common form for combined immunodeficiency disease characterized by deficient T- and B-cell function.
SCID
What are the two aims of current treatments for immunodeficiency disorders?
- Minimize and control infections
2. Replace the defective or absent components of the immune system
What are the two aims of current treatments for immunodeficiency disorders?
- Minimize and control infections
2. Replace the defective or absent components of the immune system
What is the current treatment of choice for various immunodeficiency diseases?
Bone Marrow Transplant
