Immunology Exam 4 Flashcards
Primary immunodeficiency disorders are caused by defects in the humoral immunity that include _________ & _________.
Complement
&
antibodies (B cells)
Primary immunodeficiency disorders are caused by defects in components of cellular immunity that includes _________ & ________.
T cells
&
Phagocytes
Phagocytes and complement are a part of what type of immunity?
Natural Immunity
T cells and antibodies (B cells) are a part of what type of immunity?
Acquired immunity
Immunodeficiency disorders that are X-linked affect primarily what gender?
Males
What is the primary symptom present with patients that have immunodeficiency disorders?
Recurrent infections
This B cell deficiency causes pyogenic bacterial infections - especially in the upper and lower respiratory tract.
-There is not enough or any production of antibodies to specific antigens.
Agammaglobulinemia
Immunoglobulin blood levels vary with age in normal individuals.
At birth we have low levels of ___ & ___ but adult levels of ___ because they come from the mom.
Low levels of IgA and IgM
adult levels of IgG that cross the placenta from mom
Immunoglobulin blood levels vary with age in normal individuals.
At 2-3 months, what happens to IgG?
IgG drops as maternal antibodies are metabolized
What immunodeficiency disorder usually corrects itself by 9-15 months of age?
Transient Hypogammaglobulinemia of Infancy
This immunodeficiency disorder is X-linked causing it to be seen almost exclusively in males. Development is blocked at the pro-B-cell stage. The blocked development causes no antibodies to be made.
X-linked Bruton’s Agammaglobulinemia
Cause of X-linked Bruton’s Agammaglobulinemia
Deficiency of enzyme Bruton’s tyrosine kinase
Deficiency of enzyme Bruton’s tyrosine kinase results in a failure of what not being added to heavy chain rearrangement?
Variable gene
With this immunodeficiency disorder patients may have recurrent otitis and areas of cellulitis in the diaper area. Pseudomonas aeruginosa and Staphylococcus aureus may be isolated from the skin lesions.
X-linked Bruton’s Agammaglobulinemia
What is the most common primary immunodeficiency?
IgA Deficiency
TRUE or FALSE:
Most patients with IgA deficiency are asymptomatic
TRUE
How is IgA deficiency typically discovered?
A patient with IgA deficiency forms Anti-IgA antibodies that cause anaphylaxis following a blood transfusion
What immunodeficiency is a result of having a normal number of mature B cells but they cannot differentiate into plasma cells for a variety of reasons?
Common Variable Immunodeficiency (CVI)
The following are manifestations of which immunodeficiency:
- Malabsorption
- Diarrhea
- Malignancies
- Autoimmune disorders
Common Variable Immunodeficiency
(CVI)
A patient between the ages 20 & 30 that has a low serum IgG with recurrent bacterial infections.
What immunodeficiency is this?
Common Variable Immunodeficiency
(CVI)
B cell deficiencies:
Slow development of the immune system that subsides around 2 years old
Transient hypogammaglobulinemia
B cell deficiencies:
- Development of B cells arrested at pro to pre-B cell
- Deficiency in enzyme results in failure of VH gene rearrangement
X-linked Bruton’s agammaglobulinemia
B cell deficiencies:
- Antibodies to IgA form
- Most common deficiency
- Blood transfusion anaphylaxis
IgA deficiency
B cell deficiencies:
- Development arrested at mature B cell, differentiation to plasma cells isn’t possible
- Hypogammaglobulinemia results
Common variable immunodeficiency