Immunodeficiencies Flashcards
What are immunodeficiencies?
a lack or failure of the immune system
What are primary immunodeficiencies? Secondary?
Primary = congenital Secondary = acquired
What is the most common cause of immunodeficicencies worldwide?
Malnutrition
What type of infections occur more often in a defect humoral immunity?
Extracellular bacteria
What type of infections occur more often in a defect cell mediated immunity?
Viruses and intracellular bacteria
What type of infection occur more often when there is a defect in both humoral and cell mediated immunity?
All types
Immunodeficient individuals are more susceptible to developing certain kinds of cancers. What is the etiology of these CA?
Stem from viral infections
What is X-linked agammaglobinemia?
Absence of B cells due to a defect in B cell Tyrosine kinase
What broad class of people usually gets X-linked agammaglobinemia?
Males
What type of infections are pts with X-linked agammaglobinemia susceptible to?
Pyogenic bacteria of extracellular origin
What is diagnostic for x-linked agammaglobinemia?
Absence of circulating B lymphocytes
Why can you not screen infants for x linked agammaglobinemia at 6 months?
Still have mother’s antibodies
How do you treat x-linked agammaglobinemia?
Monthyl IVIG
Why aren’t pts with x-linked agammaglobinemia more susceptible to viruses or fungal infections?
T cells intact.
What is common variable immunodeficiency?
an inability of mature B lymphocytes to differentiate into plasma cells
Does common variable immunodeficiency affect males and females equally?
Yes
How do you diagnose common variable immunodeficiency?
normal levels of circulating mature B lymphocytes which can proliferate in response to antigen, but do not differentiate into plasma cells.
Is common variable immunodeficiency acquired or congenital?
Either
What is the etiology of common variable immunodeficiency?
Unknown, but may be d/t intrinsic B cell defects
What is the treatment of common variable immunodeficiency?
monthly gamma globulin shots and antibiotic therapy PRN
What are the comorbidities of common variable immunodeficiency?
Predisposed to autoimmune diseases
What is selective IgA deficiency?
Inability to produce IgA antibodies
Is selective IgA deficiency inherited or acquired?
Either
What are the symptoms of selective IgA deficiency?
usually asymptomatic, but blood transfusions can be problematic
Why are blood transfusions problematic for pts with selective IgA deficiency?
Pts will produce anti-IgA antibodies since they were first exposed to it via breast milk. Causes hyperacute reaction
Why is selective IgA usually asymptomatic?
IgM makes up for it
Why can IgM make up for the lost IgA in selective IgA deficiency?
Has a J chain, and allows it to be transported across mucosal lining
What is the treatment for selective IgA deficiency?
Antibiotics to control infections at mucosal sites