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
Which type of immunity deficiencies have manifestations of recurrent infections with intracellular pathogens such as viruses, fungi (Candida), and intracellular bacteria?
Cellular immunity deficiency
(T cells)
TRUE or FALSE:
T cell deficiencies affect cellular immunity but can also affect humoral immunity
TRUE
Which are more difficult to manage:
Cellular immunity deficiencies
or
Humoral immunity deficiencies?
Cellular immunity deficiencies
Developmental abnormality of the third and fourth pharyngeal pouches that develop in the neck region.
DiGeorge Anomaly/Syndrome
What is missing in most patients with DiGeorge Anomaly?
Portion of chromosome 22 on the QII region
DiGeorge Anomaly causes and insufficient number of what cells? Why?
T cells - maturation occurs in the thymus but DiGeorge Anomaly affects the thymus and other neck region organs/structures
What is the treatment for DiGeorge Anomaly?
Fetal thymus and bone marrow transplant along with thymic hormones to stimulate the thymus
TRUE or FALSE:
Purine-nucleoside phosphorylase deficiency is rare autosomal recessive deficiency that can cause UTIs and Candida infections
TRUE
Which immunodeficiency causes the number of T cells to decrease as toxic purine metabolite deoxyguanine triphosphate accumulates?
Purine-nucleoside phosphorylase (PNP) deficiency
Infants with this deficiency are often confused with having neonatal HIV infection due to their symptom similarities.
PNP defieciency
What are the T-cell deficiencies discussed?
DiGeorge Syndrome/Anomaly
Purine-nucleoside phosphorylase (PNP) deficiency
What are the B-cell deficiencies discussed?
X-linked Bruton’s agammaglobulinemia
Transient hypogammaglobulinemia
Common variable immunodeficiency (CVI)
IgA deficiency
Most severe of the congenital immune deficiencies
Severe Combined Immunodeficiency
SCID
Which for of SCID is the most common?
X-linked SCID
What occurs in X-linked SCID?
- *-Normal signaling between cells is affected and halts maturation
- T cells, B cells, and NK cells are affected**
Deficiency similar to PNP. Toxic accumulation of purines in lymphoid cells impairs the proliferation of both B and T cells.
Adenosine deaminase deficiency (ADA)
Name other variety of defect grouped under SCID
-Autosomal recessive SCID: Adenosine deaminase deficiency (ADA)
Syndrome in which a patients presents with immunodeficiency, eczema, and thrombocytopenia
Wiskott-Aldrich Syndrome (WAS)
Cause of Wiskott-Aldrich Syndrome
X-linked protein defect
Severe deficiency of the naturally occurring antibodies to blood group antigens (isohemagglutinins)
Wiskott-Aldrich Syndrome (WAS)
DEFINITION:
Muscle incoordination
Ataxia
DEFINITION:
Capillary dilation
Telangiectasis
Defect in TCR and Ig gene recombination process that is autosomal recessive
Ataxia-Telangiectasia (AT)
First line of defense against infections, effective ingesting/killing, involved in antibody mediated killing.
Neutrophils
Treatment of this neutrophil defect involves granulocyte transfusions, cytokine administration, and bone marrow transplantation.
Chronic granulomatous disease (CGD)
This disease is the result of several neutrophil defects and death usually occurs in childhood typically because of infection.
Chronic granulomatous disease (CGD)
This neutrophil defect increases susceptibility to candidiasis and is the most common neutrophil abnormality. No treatment is needed in most cases of this deficiency.
Myeloperoxidase deficiency (MPO)
What causes Leukocyte adhesion molecule deficiency?
Defect in adhesion receptors for PMNs, monocytes, and T cells.
Abnormal adhesion, motility, chemotaxis, and endocytosis due to a defect in adhesion receptors
Leukocyte adhesion molecule deficiency
Delayed wound healing and chronic skin infections are manifestations of which neutrophil deficiency?
Leukocyte adhesions molecule deficiency
Hodgkin’s disease that results in the patient developing Herpes Zoster is an example of what?
Secondary immune deficiency
Which of the following are primary immunodeficiency defects:
- Chronic granulomatous disease
- SCID
- Myeloperoxidase deficiency
- DiGeorge Syndrome
- IgA deficiency
- *-SCID
- DiGeorge Syndrome
- IgA deficiency**
What are secondary immunodeficiency defects associated with? (10)
- *-Infections
- Malignancies
- Immunosuppressive agents
- Burns
- Diabetes mellitus
- Malnutrition
- Surgical procedures/trauma
- Renal malfunction
- Aging
- Alcoholic cirrhosis**
How many types of hypersensitivity are there?
4
DEFINITION:
Immune response which harms the host/self
Hypersensitivity
Immediate hypersensitivity
Type I
Antibody-Mediated Diseases
Type II
Immune Complex-Mediated Diseases
Type III
T-Cell Mediated (Delayed) Diseases
Type IV
Which of the hypersensitivities are considered immediate?
Type I
Type II
Type III
Symptoms develop within what timeframe for immediate hypersensitivities?
Minutes to hours
Which of the hypersensitivities is/are considered delayed?
Type IV
TRUE or FALSE:
Immediate hypersensitivity = Antibody mediated
TRUE
Antibody mediated hypersensitivity includes what type(s)?
Type I, II, and III
Cell-mediated hypersensitivity includes what type(s)?
Type IV
Symptoms develop within what timeframe for delayed hypersensitivity?
24 - 48 hours after contact with antigen
TRUE or FALSE:
Delayed hypersensitivity = Cell mediated
TRUE
What other name is type I hypersensitivity known as?
Anaphylactic
What is another name for allergens?
Atopic antigens
DEFINITION:
Substances that trigger the formation of IgE
Atopic antigens
DEFINITION:
Inherited tendency to respond to naturally occurring allergens with continued production of IgE
Atopy
Old name for IgE
Reagin
Which T cell regulates IgE production?
Th2
Which hypersensitivity:
- Allergen activates B cells to form IgE secreting plasma cells
- IgE antibodies bind to high-affinity receptors on mast cells and coat the cell (sensitization)
- Subsequent exposure to the same allergen leads to crosslinking of bound IgE antibodies on mast cells
- Results in the release of granules from mast cell
- Chemical mediators are released
Type I hypersensitivity
TRUE or FALSE:
Asthma is a Type II hypersensitivity
FALSE
Type I
Primary mediator in Type I hypersensitivity
Histamine
Secondary mediators for Type I hypersensitivity (4)
- *-Prostaglandins
- Leukotrines
- Platelet activating factor (PAF)
- Cytokines**
Symptoms that occur with Type I hypersensitivity
- *-Wheal and flare
- Asthma
- Anaphylaxis
- Skin eruptions**
Testing methods for Type I hypersensitivity
- *-RIST
- RAST
- Skin test**
Test for total IgE
RIST: radioimmunosorbent test
Test for level of specific IgE
RAST: radioallergosorbent test
RIST is what type of test?
Sandwich EIA - competitive
RAST is what kind of test method?
ELISA microarray test