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Quiz 3 Pathology > Immunopathology IV + CPC > Flashcards

Immunopathology IV + CPC Flashcards

1
Q

Name the X linked immunodeficiency syndromes

A

X-linked agammaglobulinemia
Hyper-IgM syndrome
Severe combined immunodeficiency
Wiskott-Aldrich syndrome

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2
Q

Name the autosomal dominant primary immunodeficiency syndromes

A

C1 inhibitor deficiency

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3
Q

Name the recessive immunodeficiency syndromes

A

DiGeorge syndrome

SCIDS

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4
Q

Describe the pathology of X-linked agammaglobulinemia

A

Failure of B cell precursors to develop into mature B cells

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5
Q

What is mutated in X-linked agammaglobulinemia?

A

Mutations in bruton tyrosine kinase gene which is required for signal transduction for Ig light chain rearrangement

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6
Q

What kinds of infections will you see with X-linked agammaglobulinemia?

A

Recurrent bacterial infections
Enteroviral encephalitis
Giardia

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7
Q

What are the symptoms of x linked agammaglobulinemia?

A

Absent B Cells
Decreased serum immunoglobulin
Normal marrow B cell precursors
Normal T-cell mediated reactions

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8
Q

What is common variable immunodeficiency?

A

Characterized by hypogammaglobulinemia

Normal B cell count, but unable to differentiate into plasma cells

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9
Q

What is isolated IgA deficiency

A

Low levels of serum and secretory IgA

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10
Q

What are the complications arising form isolated IgA deficiency?

A

causing increased risk of respiratory and gastrointestinal disorders
anaphylactic transfusion reaction

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11
Q

What is the cause of hyper IgM syndrome?

A

Defect in ability of helper T cells to deliver activating signals to B cells

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12
Q

What would you observe in a hyper IgM syndrome?

A

Lots of IgM antibodies but deficiency of IgG, IgA, and IgE. etc.

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13
Q

Is Hyper IgM linked?

A

The majority of hyper IgM cases are X linked

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14
Q

How do you treat humor immunodeficiency syndromes?

A

With IvIg

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15
Q

What is DiGeorge syndrome?

A

Failure of development of 3rd and 4th pharyngeal pouches during embryogenesis which causes thymic hypoplasia and loss of T cell immunity

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16
Q

Where is the deletion for DiGeorge Syndrome?

A

22q11 deletion

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17
Q

What infections are associated with T cell defects?

A

Bacterial sepsis
CMV, epstein barr, severe chicken pox
chronic respiratory/intestinal infection
Candida

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18
Q

What do you observe in severe combined immunodeficiency?

A

Low T cells and also low B cells with hypogammaglobulinemia. Prevents differentiation of stem cells into lymphocytes

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19
Q

What causes SCID?

A

cytokine receptor deficiency (impacts T cell development and is X-linked)
OR
ADA deficiency impacts immature lymphocytes (autosomal recessive)

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20
Q

How do you test for the presence of autoimmune disorders?

A

Isohemmagglutinin testing. These isohemagglutinins cross react with A or B

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21
Q

What is Wiskott-Aldrich syndrome?

A

X linked recessive disorder causing low IgM, normal IgG, elevated IgA and IgE
Causes thrombocytopenia, eczema and immune deficiency

22
Q

What is the most common type of complement deficiency?

A

C2 deficiency

23
Q

What are the symptoms of C2 deficiency?

A

No increased risk of infection, but there is an increased risk of SLE like autoimmune disease

24
Q

What are the symptoms of a C3 deficiency?

A

Increased risk of bacterial infection

25
Q

What are the symptoms of a C5-9 deficiency?

A

Increased susceptibility to neisseria

26
Q

What does C1 inhibitor deficiency look like?

A

Episodic edema of skin and mucosal surfaces due to stressor trauma. Caused by unregulated C1

27
Q

What are secondary causes of immunodeficiency?

A 
Chemotherapy
Cancer
AIDS (infections)
Renal disease
Diabetes
28
Q

What is used to diagnose HIV?

A

p24 capsid antibodies

29
Q

What are the products yielded by HIV viral protease?

A

gag and pol

30
Q

When can you detect HIV viremia?

A

Less than 1 week after primary infection

31
Q

Describe how cells are infected by HIV

A

gp120 binds CD4 receptor and chemokine co-receptors (CCR5 or CXCR4) on T cells, monocytes, macrophages, or dendritic cells

This induces a conformational change in gp41 causing fusion

32
Q

When can seroconversion be detected for HIV?

A

3-7 weeks after initial infection

33
Q

What feature of HIV prevents antibody binding?

A

glycan shield

34
Q

Why does a high mutation rate aid in the evasion of host cell defenses?

A

Small changes to sugars and positions prevent detection

35
Q

Describe the clinical course of HIV

A
  1. Initial infection+virema+CTLs rise
  2. Antibody to envelope develops
  3. Antibody to p24 develops
  4. Cd4 cells decline steadily
36
Q

What are category B HIV infections?

A

Yeast
PID
Hairy cell leukemia
Shingles

37
Q

What are category C AIDs conditions?

A

cytomegalovirus
toxoplasma
pneumocystis
kaposi sarcoma

38
Q

What are the highest risk groups for HIV?

A

male to male sexual contact
heterosexual female contact
heterosexual male contact
IV drug use

39
Q

What does gag part of the HIV chromosome do?

A

Formation of the capsid protein

40
Q

What does the pol part of the HIV chromosome do?

A

Reverse transcriptase

41
Q

How would you confirm the presence of amyloid?

A

Through applying congo red dye stains, which turn green under polarized light

42
Q

The fibrils in primary amyloidosis are composed of:

A

kappa or gamma light chains

43
Q

The fibers in secondary amyloiosis are composed of:

A

protein A

44
Q

How does amyloid cause disease?

A

By crowding out normal tissue elements preventing normal function

45
Q

Where would you find an AB amyloid?

A

CNS only

46
Q

What is the structure of amyloid?

A

beta pleated sheets conformation

47
Q

Describe the three main types of amyloid:

A

AL (light chain): Derived from Ig light chains
AA (amyloid-associated): “acute phase” amyloid. Associated with chronic inflammation.
AB: Produced from beta amyloid intermediate found in alzheimer’s disease.

48
Q

What are other amyloid proteins?

A
  1. Mutant transthyretin. Binds and transports thyroxine and retinol
  2. Beta-2 microglobulin in hemodialysis patients
49
Q

What causes deposition of AL type amyloids?

A

Immunocyte dyscrasias

50
Q

Reactive systemic amyloidosis–what kind of fibrils?

A

AA protein deposition. associated with chronic inflammation like RA, inflammatory bowel disease, etc.

51
Q

Hemodialysis associated amyloiosis:

A

Beta-2 microglobulin deposition

Quiz 3 Pathology (5 decks)

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