Cohen lectures

Question 1

What is the normal range of WBCs?

Answer 1

1100-5500/uL (20-50% of total WBCs)

Question 2

What should you do to blood before giving it to an immunodeficient patient?

Answer 2

Irradiate it

Question 3

What is the major immunologic deficiency in DiGeorge Syndrome?

Answer 3

Absence of a thymus so unable to make mature T-lymphocytes.

Question 4

What are other physiological characteristics of DiGeorge syndrome? (Hint: CATCH-22)

Answer 4

C-Cardiovascular abnormalities A-Abnormal face T-Thymus absent C-Clefts H-Hypocalcemia/hypoparathyroid 22-Abnormality on the 22 chromosome

Question 5

What is the major immunologic deficiency in SCID?

Answer 5

A bone marrow precursor fails to produce functioning B and T cells.

Question 6

What are the 3 genetic causes of SCID discussed in class?

Answer 6

1.) X1 - X linked recessive in the gamma chain that forms a part of the IL-2R structure deficiency or mutation which prevents proper maturation 2.) Adenosine deaminase deficiency leading to excess adenosine deaminase 3.) A mutation that affects V(D)J recombination

Question 7

What is the major immunologic deficiency in X-linked (Bruton) hypogammaglobulinemia?

Answer 7

Lack of mature B cells because B cell precursors lank a kinase that allows them to make antibody.

Question 8

What is the major immunologic deficiency in Common Variable Immunodeficiency?

Answer 8

Cyclic trouble making specific antibodies

Question 9

What is the major immunologic deficiency in Hyper IgM syndrome?

Answer 9

Inability to class-switch from IgM to IgG

Question 10

What is the most common immunodeficiency?

Answer 10

Selective IgA deficiency

Question 11

What are the two most common characteristics of selective IgA deficiency?

Answer 11

Chronic allergies and GI problems

Question 12

What infections are most likely to be seen in patients with Brutons?

Answer 12

Infections associated with mucous membranes (pneumonia and chronic diarrhea, enteroviruses). Polio from the oral polio vaccine

Question 13

What infections are T cell deficiencies commonly associated with?

Answer 13

Severe infections with intracellular pathogens, especially Candida albicans and pneumocystis jirovecii

Question 14

What infections are B cell deficiencies commonly associated with?

Answer 14

Infections with extracellular pathogens such as stap aureus, H. influenzae, and strep pneumonia.

Question 15

Which syndromes will have increased susceptibility to intracellular pathogens?

Answer 15

SCID, DiGeorge

Question 16

Which syndromes will have increased susceptibility to extracellular pathogens?

Answer 16

Brutons, Common Variable Immunodeficiency, Hyper IgM syndrome, Selective IgA deficiency

Question 17

What is wrong with nude mice?

Answer 17

No thymus

Question 18

What are two treatments for ADA deficient SCID patients?

Answer 18

1.) ADA given as a drug 2.) Bone marrow transplant 3.) Gene therapy

Question 19

What is the risk of gene therapy discussed in class?

Answer 19

May insert in the wrong place and promote the development of cancer

Question 20

What is the treatment for DiGeorge syndrome?

Answer 20

Thymus transplant - from fetal thymus or thymic stromal cells

Question 21

What is the risk of tissue transplant in immunodeficient patients?

Answer 21

Graft verus host disease

Question 22

What are the initial tests done to determine B cell deficiency?

Answer 22

Serum protein electrophoresis, quatitiative IgG IgM IgA levels, specific Abs prior to immunization, presence of ABO isohemagglutins

Question 23

What are the advanced tests done to determine B cell deficiency?

Answer 23

Ab response to novel Ags, sequence specific genes

Question 24

What are the initial tests done to determine T cell deficiency?

Answer 24

Skin test with recall Ab panel, total lymphocytes count

Question 25

What are the advanced tests done to determine T cell deficiency?

Answer 25

CD3 CD4 CD8 counts. Mitogen response, MLR, cytokine measurements

Question 26

What are the initial tests done to determine phagocytes deficiency?

Answer 26

WBC count, differential, morphology, NTB test and oxidative burst

Question 27

What are the advanced tests done to determine phagocytes deficiency?

Answer 27

Assay for phagocytosis, genetics

Question 28

What are the initial tests done to determine complement deficiency?

Answer 28

CH50, Assay for C1inh

Question 29

What are the advanced tests done to determine complement deficiency?

Answer 29

Individual complement component levels

Question 30

How can commercial gamma globulin be given?

Answer 30

IVIG (more common) or SCIG

Question 31

What is in commercial gamma globulin?

Answer 31

Mostly (99%) mixed IgG from multiple donors

Question 32

Name the viruses given by Cohen that are immunosuppressive in humans?

Answer 32

HIV, measles, mono, CMV

Question 33

How is tissue damaged in a Type II autoimmune reaction?

Answer 33

Antibody mediated IgG, IgM, IgA

Question 34

Name two diseases that result from ?stimulatory hypersensitivity.?

Answer 34

Graves Disease - hyperthyroidism resulting from Ab to TSH (called long-acting thyroid stimulator). Inappropriate tachycardia - Ab to beta-adrenergic receptor. Both stimulate the action of the cells they target.

Question 35

T or F: ?Lumpy-bumpy? immunofluorescence pattern is characteristic of Type II immunopathology.

Answer 35

False. Type III

Question 36

T or F: ?Linear? immunofluorescence pattern is characteristic of Type II immunopathology.

Answer 36

TRUE

Question 37

What is Dressler syndrome?

Answer 37

Post-cardiac injury syndrome. When a person makes autoantibody to heart tissue post-surgery. Resolves over time with anti-inflammatories.

Question 38

What is Goodpasture syndrome?

Answer 38

Development of autoantibodies to lung and kidney basement membranes, but injury more commonly seen only in the kidney unless the person is a smoker.

Question 39

T or F: Goodpasture syndrome is a type III immunopathology.

Answer 39

False. Type II, antibodies are directed against basement membranes, not just trapped inside them.

Question 40

T or F: Autoimmune thrombocytopenia purpura is due to complement mediated destruction.

Answer 40

TRUE

Question 41

What is rheumatic heart disease? rheumatic fever?

Answer 41

Autoimmune condition acquired after repeat streptococcus infection. Antibody against streptococcus cross reacts with laminin in the heart. R fever is more widespread to skin and CNS.

Question 42

What is the most common autoimmune disease?

Answer 42

Rheumatoid arthritis

Question 43

What is rheumatic factor?

Answer 43

IgM anti-IgG. Discovered in RA patient?s serum, although might not actually cause the disease.

Question 44

What does the Aire gene do?

Answer 44

The Aire gene up regulates expression of many body genes to weed out self-reactive T cells. In some families, there is a mutation of the alpha chain of the T cell that prevents it from reacting to Aire. This results in release of auto reactive T cells.

Question 45

T or F: A forbidden clone is when you make a clone of yourself for sexual purposes.

Answer 45

False, but if only it was true! The emergence of a clone that escapes clonal abortion.

Question 46

T or F: Cross-reaction is what happens to vampires when they enter a church.

Answer 46

False. It is when an antibody reacts to both foreign antibody and self.

Question 47

T or F: Illicit help is when your dad and the maid play games in the bedroom when mommy isn?t home.

Answer 47

False. It is when a B-cell ingests both self and foreign peptide and when activated by a Tfh makes antibodies to self peptide.

Question 48

What is passive antibody?

Answer 48

Antibody from another person that reacts to the donor?s cells (ex. hemolytic disease of the newborn).

Question 49

What is sequestered antigen?

Answer 49

When an antigen that is normally sequestered safe in the tissue is exposed.

Question 50

Resection of which organ can improve myasthenia gravis in some patients?

Answer 50

Thymus