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Blood and lymph Unit II > Cohen lectures > Flashcards

Cohen lectures Flashcards

1
Q

What is the normal range of WBCs?

A

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

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2
Q

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

A

Irradiate it

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3
Q

What is the major immunologic deficiency in DiGeorge Syndrome?

A

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

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4
Q

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

A

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

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5
Q

What is the major immunologic deficiency in SCID?

A

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

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6
Q

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

A

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

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7
Q

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

A

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

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8
Q

What is the major immunologic deficiency in Common Variable Immunodeficiency?

A

Cyclic trouble making specific antibodies

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9
Q

What is the major immunologic deficiency in Hyper IgM syndrome?

A

Inability to class-switch from IgM to IgG

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10
Q

What is the most common immunodeficiency?

A

Selective IgA deficiency

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11
Q

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

A

Chronic allergies and GI problems

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12
Q

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

A

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

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13
Q

What infections are T cell deficiencies commonly associated with?

A

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

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14
Q

What infections are B cell deficiencies commonly associated with?

A

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

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15
Q

Which syndromes will have increased susceptibility to intracellular pathogens?

A

SCID, DiGeorge

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16
Q

Which syndromes will have increased susceptibility to extracellular pathogens?

A

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

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17
Q

What is wrong with nude mice?

A

No thymus

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18
Q

What are two treatments for ADA deficient SCID patients?

A

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

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19
Q

What is the risk of gene therapy discussed in class?

A

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

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20
Q

What is the treatment for DiGeorge syndrome?

A

Thymus transplant - from fetal thymus or thymic stromal cells

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21
Q

What is the risk of tissue transplant in immunodeficient patients?

A

Graft verus host disease

22
Q

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

A

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

23
Q

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

A

Ab response to novel Ags, sequence specific genes

24
Q

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

A

Skin test with recall Ab panel, total lymphocytes count

25
Q

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

A

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

26
Q

What are the initial tests done to determine phagocytes deficiency?

A

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

27
Q

What are the advanced tests done to determine phagocytes deficiency?

A

Assay for phagocytosis, genetics

28
Q

What are the initial tests done to determine complement deficiency?

A

CH50, Assay for C1inh

29
Q

What are the advanced tests done to determine complement deficiency?

A

Individual complement component levels

30
Q

How can commercial gamma globulin be given?

A

IVIG (more common) or SCIG

31
Q

What is in commercial gamma globulin?

A

Mostly (99%) mixed IgG from multiple donors

32
Q

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

A

HIV, measles, mono, CMV

33
Q

How is tissue damaged in a Type II autoimmune reaction?

A

Antibody mediated IgG, IgM, IgA

34
Q

Name two diseases that result from ?stimulatory hypersensitivity.?

A

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.

35
Q

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

A

False. Type III

36
Q

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

A

TRUE

37
Q

What is Dressler syndrome?

A

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

38
Q

What is Goodpasture syndrome?

A

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

39
Q

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

A

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

40
Q

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

A

TRUE

41
Q

What is rheumatic heart disease? rheumatic fever?

A

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.

42
Q

What is the most common autoimmune disease?

A

Rheumatoid arthritis

43
Q

What is rheumatic factor?

A

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

44
Q

What does the Aire gene do?

A

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.

45
Q

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

A

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

46
Q

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

A

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

47
Q

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

A

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

48
Q

What is passive antibody?

A

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

49
Q

What is sequestered antigen?

A

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

50
Q

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

A

Thymus

Blood and lymph Unit II (6 decks)

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