Immunology: Test 2: 3. Immunodeficiency

Front 1

what is a primary immunodeficiency

Back 1

Due to intrinsic (congenital or acquired) defects in cells or factors of the immune system

Front 2

what is a secondary immunodeficiency

Back 2

Result from extrinsic factors, such as drugs, irradiation, malnutrition, or infection (e.g., AIDS)

Front 3

what is the number one cause of immune deficits in the world

Back 3

malnutrition

Front 4

please list a few of the most common GENERAL primary immunodeficiencies (there are 5 but i'd be happy with 3)

Back 4

a) B-cell deficiencies
b) T-cell deficiencies
c) Combined T and B-cell deficiencies
d) Defects in Phagocytes
e) Complement deficiencies

Front 5

if you have an IgA deficiency, what type of immunodeficiency do you have?

Back 5

Antibody (B-cell) deficiency

Front 6

What is the most common immunodeficiency?

Back 6

IgA deficiency

Front 7

what symptoms do you see with IgA deficiency

Back 7

Recurrent bacterial infections or asymptomatic

Front 8

What is X-linked hypogammaglobulinemia?

Back 8

no B-cells
affects male children
victim prone to recurrent bacterial infections

Front 9

what is IVIG? used to treat?

Back 9

intravenous immunoglobin

used to treat hypogammaglobulinemia

Front 10

What is Immunodeficiency with hyper-IgM

Back 10

VERY RARE

large amount of serum IgM, with deficient production of IgG and IgA

problem with T-cell, not able to do isotype switching

Front 11

What is the genetic defect seen in immunodeficiency with hyper-IgM?

Back 11

in the CD40 (CD154) ligand on T cells, which must bind on B cells to initiate antibody class switch from IgM to IgG/A

Front 12

What is Common Variable Immunodeficiency (CVI)

Back 12

normally late onset

•Recurrent sinopulmonary infections along with other systems involved
•Slow decline in total serum immunoglobulin
•Increased incidence of autoimmune disease, leukemia and lymphoma
•Defect in B cell development

Front 13

what type of cells are missing from patients with CVI?

Back 13

no plasma cells

Front 14

John was born at term via a normal delivery. At age 6 months he developed URT infection with complications. One-month later he was hospitalized with acute otitis media that developed into bacterial meningitis. John is now 2 years old and has been hospitalized a total of 5 times with severe infections. Lab results show very low, almost undetectable levels of B cells. Serum antibody concentrations (IgG, IgM, and IgA) were also extremely low.
What is the most likely cause of John’s lack of resistance to infection?

A. IgA deficiency
B. X-linked hypogammaglobulinemia
C. IgG2 subclass deficiency
D. Common variable immunodeficiency

Back 14

X-linked hypogammaglobulinemia

Front 15

What is DiGeorge Syndrome? (Congenital Thymic Aplasia),

Back 15

• The thymus and parathyroids fail to develop.
• The T cell deficiency is variable depending on how badly the thymus gland is affected.
•Often presents as the triad of: a) Hypocalcemia b) Diminished T-cell function
c) Congenital heart disease.

Front 16

if you see a person with : a) Hypocalcemia b) Diminished T-cell function
c) Congenital heart disease.

what do they have?

Back 16

DiGeorge Syndrome (Congenital Thymic Aplasia)

Front 17

what is X linked SCIDs?

Back 17

Most commonly occurring form
Mutated cytokine receptor gamma chain gene, gammac
Impaired lymphocyte development

Front 18

in SCIDS, you see deficiencies in what two enzymes?

Back 18

in adenosine deaminase (ADA) or purine nucleoside phosphorylase (PNP)

Front 19

What is Wiskott-Aldrich Syndrome?

Back 19

Immunodeficiency with eczema, thrombocytopenia, and recurrent infections
X-linked inheritance
Elevated serum IgA and IgE but decreased IgM
* Defective T cell function and appearance

Front 20

which immunodificiency will show eczema and thrombocytopenia?

Back 20

Wiskott-Aldrich Syndrome

Front 21

If you have abnormally small platelets, and lowered number, what disease you thinkin sucka?!

Back 21

Wiskott-Aldrich Syndrome

Front 22

Hi my name is

Back 22

chicka chicka slim shady

Front 23

Elevated serum IgA and IgE and decreased IgM are associated with what?

Back 23

Wiskott-Aldrich Syndrome

Front 24

what is an extrinsic defect in phagocytosis?

Back 24

defects in some other aspect of the immune or inflammatory response that diminish phagocytosis

Front 25

what is an intrinsic defect in phagocytosis?

Back 25

involve defects in the intracellular killing machinery, e.g. in Chronic granulomatous disease.

Front 26

what is chronic granulomatous disease?
age of onset?
genetics?
problem?

Back 26

Age of onset 2 years
Most common form is X-linked
Defects in intracellular killing
Recurrent or persistent infections

Defect in phagocyte function

Front 27

What defect is there in granulomatous disease?

Back 27

NADPH oxidase

which generates free radicals used to destroy the foreign microorganism

Front 28

how can you treat granulomatous disease?

Back 28

give interferon gamma

turns on more of NADPH oxidase activity

Front 29

what is Leukocyte adhesion deficiency type I? defect in what? symptoms?

Back 29

β2 integrin defect
Severe periodontitis, early tooth decay, recurrent infections
High blood PMN count
High mortality

Front 30

what might you be afflicted with if you had a B2 integrin defect?

Back 30

Leukocyte adhesion deficiency type I

Front 31

complement deficiency can lead to what?

Back 31

autoimmune disease

Front 32

What is Hereditary Angioedema (HAE)?

Back 32

Deficiency of C1INH

remember: ). C1INH actually inhibits activated Hageman factor and thus regulates enzymes involved in complement, kinin generation, the clotting system, and fibrinolysis

recurrent attacks of edema of subcutaneous and submucosal tissues, with typical attacks last 1-4 days without serious effect.

Front 33

What time is it?

Back 33

GAME TIME

WHOOP!

Front 34

If you suspect immunodeficiency, should you give live vaccines?

Back 34

nooooooo!