Scnm Q5 Pathology Quiz 2

Front 1

Describe Type I Hypersensitivity

Back 1

Anaphylactic. Occurs WITHIN MINUTES after antigen binds to antibody on MAST CELLS (or basophils). Mediated by IgE

Front 2

What type of cell stimulates B lymphocytes to produce IgE?

Back 2

Th2 CD4+ Thelper cells.

Front 3

Describe the 2 phases that occur in a Type I hypersensitivity response?

Back 3

1) Initial response: Starts 5-30 minutes after exposure to allergen, causes vasodilation, vascular leakage, smooth muscle spasm, glandular secretions
2) Late Phase Response: Starts 2-8 hours later without exposure to allergen. Causes infiltration of tissues with eosinophils, basophils, monocytes, which cause damage to the mucosal epithelial cells.

Front 4

What binds to MHC Class I? What binds to MCH Class II?

Back 4

MHC Class I - CD8+ T cells
MHC Class II - CD4+ T cells

Front 5

What surface ligand on T cells (which normally attaches to a receptor on B cells) gets mutated in X-linked hyper IgM syndrome?

Back 5

CD40. There is a CD40 receptor on B cells that binds the CD40L on T cells and is required to trigger Ig class switching.

Front 6

What are the 3 types of dendritic cells and where are they found?

Back 6

1) Dendritic - lymphoid tissues, heart & lung
2) Langerhans cells - epidermis
3) Follicular dendritic cells - spleen & lymph nodes

Front 7

Which cells express MHC Class I? Which cells express MHC Class II?

Back 7

All nucleated cells express MHC Class I. All Antigen Presenting Cells (macrophages, dendritic cells, B-cells) express MHC Class II.

Front 8

Which immunoglobulins mediate Type II hypersensitivity (cytotoxic)?

Back 8

Mostly IgG and IgM. However, for targets that are too large to be phagocytosed, IgE and eosinophils are involved (ex: parasites).

Front 9

Goodpasture, Graves, Rheumatic Fever and Myasthenia Gravis are all examples of what type of hypersensitivity reaction?

Back 9

Type II, cytotoxic hypersensitivity.

Front 10

Systemic lupus erythematosus (SLE) and Arthus reaction are examples of what type of hypersensitivity reaction?

Back 10

Type III - immunocomplex hypersensitivity. Depositions of antigen-antibody complexes cause pathology. SLE is an example of DNA - anti-DNA.

Front 11

What is Type IV hypersensitivity and what are some examples?

Back 11

Type IV hypersensitivity is cell-mediated. It does not require the participation of antibodies. It is mediated by T cells. Some examples are: contact dermatitis, MS

Front 12

What test results are normally seen in SLE?

Back 12

Positive ANA
Anti-double-stranded DNA antibodies
Anti-Sm (smith) antigen
False positive test for syphilis

Front 13

What are the common clinical features of SLE?

Back 13

1) Malar rash
2) Photosensitivity
3) Polyarthalgia - Pain in peripheral joints (w/out deformity)
4) Normocytic anemia
5) Glomerulonephritis
6) Fever, weight loss, fatigue

Front 14

What are the symptoms of Discoid Lupus Erythematosus (DLE)?

Back 14

Mainly just malar rash.

Front 15

Who normally gets Sjogren Syndrome?

Back 15

Women, around 50 yrs old.

Front 16

What are the major symptoms of primary Sjogren Syndrome?

Back 16

Keratoconjuctivitis sicca (dry eyes)
Xerostomia (dry mouth)

Front 17

What antibodies are seen in Sjogren syndrome?

Back 17

Anti-SS-A and Anti-SS-B. SS-A and SS-B are ribonuclear proteins.

Front 18

What type of cancer are people with Sjogren Syndrome at increased risk of contracting?

Back 18

Malignant lymphoma.

Front 19

What is the pathology of Sjogren Syndrome?

Back 19

Lymphoid infiltrates destroy acini and ducts of secretory glands. Type II and Type IV reaction involved.

Front 20

What are the autoantibodies involved in Scleroderma?

Back 20

ANA (90% of the time)
Scl 70 (70% of the time - this is a non-histone nuclear protein)
Rheumatoid factor (34% of the time)

Front 21

What antibody is associated with a more benign variant of Scleroderma called CREST syndrome?

Back 21

Antibody to centromere

Front 22

What are some of the clinical signs of Scleroderma?

Back 22

-No hair on hands
-Skin is tight and shiny
-Face is tight without expression
-Cheilosis at the corners of the mouth from riboflavin deficiency due to malabsorption that can occur
-Dysphagia (trouble swallowing) due to fibrosis of the lower 2/3 of esophagus
-Raynaud phenomenon (ischemia of the fingers, pallor, paresthesia, pain)
-Polyarthralgia

Front 23

What is the pathology of Scleroderma?

Back 23

Endothelial injury results from serum cytotoxic factors, autoimmunity or toxins.
Fibroblast proliferation causes excess production of collagen in many tissues: dermis, blood vessels, lungs, GI tract, kidneys, heart

Front 24

What are the clinical findings of CREST syndrome?

Back 24

C - calcification, centromere antibody
R - Raynaud's phenomenon
E - Esophageal dysmotility
S - sclerodactyly (tapered, claw-like fingers)
T - telangiectasias (blood vessel dilations)

Front 25

What differentiates Polymyositis from Dermatomyositis?

Back 25

PM: no skin involvement
DM: has skin involvement

Front 26

Polymyositis/Dermatomyositis occurs primarily in whom?

Back 26

Women aged 40-60 years, common in African-Americans

Front 27

What are the common clinical findings in Polymyositis/Dermatomyositis?

Back 27

1) Bilateral proximal muscle weakness (difficulty rising from chairs, climbing stairs or raising both hands above head)
2) Heliotrope eyelids (raccoon eyes)

Front 28

In what autoimmune disease is "shawl sign" seen?

Back 28

Dermatomyositis

Front 29

What are the lab findings in Polymyositis/Dermatomyositis?

Back 29

1) Serum ANA is positive in < 30% cases
2) Increased serum creatinine kinase
3) Jo-1 antibodies
4) Muscle biopsy shows a lymphocytic infiltrate

Front 30

Gottron's Papules on the distal interphalangeal joints is found in what pathology?

Back 30

Dermatomyositis

Front 31

What T-cell immunodeficiency disorder is caused by the 3rd and 4th pharyngeal pouches failing to develop into the thymus and parathyroid glands?

Back 31

DiGeorge Syndrome

Front 32

What are the clinical features of DiGeorge Syndrome?

Back 32

1) Congenital heart defect
2) Hypocalcemia (due to lack of PTH) which can cause tetany & other problems
3) Chronic viral, fungal, bacterial and protozoal infections

Front 33

T/F: Bruton's hypogammaglobulinemia is an autosomal recessive disorder.

Back 33

False. It is an X-linked recessive disorder.

Front 34

What types of cells are effected in Bruton's hypogammaglobulinemia?

Back 34

It is a B-cell deficiency disorder

Front 35

What are the characteristic signs/symptoms of Bruton's hypogammaglobulinemia?

Back 35

1) Recurrent pyogenic infections of upper respiratory tract
2) Severe hypogammaglobulinemia
3) Absence of mature B cells and plasma cells in circulation
4) Germinal centers of lymph nodes are underdeveloped

Front 36

What causes Bruton's hypogammaglobulinemia (also known as X-linked agammaglobulinemia)?

Back 36

X-linked recessive disorder that mutates a tyrosine kinase, resulting in the failure of pre-B cells to become mature B cells

Front 37

What is the pathology behind Common Variable Immunodeficiency?

Back 37

Although there are a NORMAL number of circulating B cells, there is an ABSENCE of PLASMA CELLS.
This is caused by either the T cells sending no activation signal to B cells or because of an intrinsic B cell defect.

Front 38

What are the clinical features of Isolated IgA Deficiency?

Back 38

Since there are extremely low levels of IgA, mucosal defenses are weakened causing increased:
Sinopulmonary infections
IgE-mediated allergies
GI infections
There is also increased frequency of SLE and RA

Front 39

Is SCID (Severe Combined Immunodeficiency) a deficiency in T-cells, B-cells, or both?

Back 39

Both

Front 40

What are the clinical features of SCID (Severe Combined Immunodeficiency)?

Back 40

1) Appears around 6 months of age
2) Recurrent infections
3) Failure to thrive
4) Death within 1 year without bone marrow transplant

Front 41

What is the only acquired immunodeficiency disease?

Back 41

AIDS

Front 42

Which cells does HIV affect?

Back 42

There is infection and severe loss of CD4+ T cells. It also affects macrophages and dendritic cells.

Front 43

Besides CD4+ T cells, what other cells become damaged in HIV?

Back 43

Macrophages and dendritic cells

Front 44

What are the phases from HIV infection to AIDS?

Back 44

1) Acute phase - mononucleosis-like syndrome 3-6 weeks after infection (sore throat, myalgias, fever, fatigue) but resolve 2-4 weeks later
2) Latent phase: Asymptomatic or they develop thrombocytopenia, lymphadenopathy, thrush or herpes zoster; lasts 7 to 10 years after infection; CD4 T cell count over 500 cells/mm3; Cytotoxic T cells control but do not clear HIV reservoirs in dendritic cells
3) Early symptomatic phase: CD4 T cell count 200 to 500 cells/mm3; fatigue, fever, infections, secondary neoplasms, encephalitis
4) AIDS: fewer than 200 CD4+ T cells

Front 45

What is the most common lung infection in AIDS?

Back 45

Pneumocystis carinii

Front 46

What are 2 of the most common neoplasms for people with AIDS?

Back 46

1) Kaposi sarcoma
2) Lymphomas of B cell origin

Front 47

What are the differences between benign tumors and malignant tumors?

Back 47

Benign tumors:
1) do not invade adjacent tissues
2) do not metastasize
3) are more differentiated
4) resemble their tissue of origin
Malignant tumors:
1) invade other tissues
2) metastasize to distant sites

Front 48

What is the difference between sarcomas and carcinomas?

Back 48

Sarcoma: malignant tumors of mesenchymal origin
Carcinoma: malignant tumors of epithelial cells

Front 49

Most malignant tumors end in "sarcoma" or "carcinoma" per the naming convention. What are the exceptions?

Back 49

Hepatoma, melanoma, seminoma, lymphoma, leukemia

Front 50

What are some typical features of the nucleus of a malignant tumor?

Back 50

Enlarged, hyperchromatic nuclei
Clumped chromatin
Mitoses have normal and atypical mitotic spindles

Front 51

What is different about the mitotic activity of cancer cells?

Back 51

Normally about 1.5% of cells are dividing. In cancer it can be 20%.

Front 52

What are the 3 pathways that cancer can use to metastasize?

Back 52

1) Direct seeding (ex: cancer of the ovaries grows into peritoneal cavity)
2) Lymphatic spread
3) Hematogenous spread

Front 53

Which type of cancer generally uses lymphatic spread?

Back 53

Usually carcinomas, but can be sarcomas.

Front 54

Which type of cancer generally uses hematogenous spread and which organs are the most common site of metastasis?

Back 54

Usually sarcomas, but can be carcinomas. Liver and lungs are common sites because all portal area drainage flows to liver and all caval blood flows to lungs.

Front 55

Which oncogene is associated with breast and ovarian carcinomas?

Back 55

BRCA-1

Front 56

Which oncogene is associated with retinoblastomas, osteosarcomas and small cell lung carcinomas?

Back 56

Rb

Front 57

What is the ras gene and why is it significant in cancer?

Back 57

Ras is the single most common abnormality of dominent oncogenes in human tumors; it's present in 30% of tumors.
The ras protein normally binds GDP and remains inactive until a growth factor stimulates it to bind GTP and become active.

Front 58

What is the chromosomal translocation involved in Burkitt's Lymphoma?

Back 58

C-myc gene of chromosome 8 is translocated to chromosome 14

Front 59

What is the chromosomal translocation involved in chronic myeloid leukemia?

Back 59

C-abl gene on chromosome 9 is translocated to the bcr gene on chromosome 22 (Philadelphia chromosome)

Front 60

Which gene is considered the "gatekeeper" and what does it do?

Back 60

APC (Adenomatous Polyposis Coli). It prevents nuclear transcription. It's called the gatekeeper because it's critical that this gene gets mutated first. Associated with familial polyposis (colorectal carcinoma).

Front 61

Which genes are considered "caretakers" and what does they do?

Back 61

Mismatch repair genes, BRCA1, BRCA2, p53

Front 62

What are the growth factors that stimulate angiogenesis?

Back 62

VEGF and bFGF

Front 63

Angiogenesis is inhibited by what?

Back 63

p53

Front 64

What is E-cadherin?

Back 64

E-cadherin is a family of transmembrane glycoproteins that adhere the normal cells to each other.

Front 65

What are catenins?

Back 65

Catenins link E-cadherin with cell cytoskeleton.

Front 66

Why is CD44 important in cancer formation?

Back 66

CD44 adhesion molecule or its variants is important for the adhesion of tumor cells to the lymph nodes.

Front 67

What cancers are associated with EBV?

Back 67

Burkitt's lymphoma, nasopharyngeal carcinoma

Front 68

What cancers are associated with HPV?

Back 68

Squamous cell carcinoma of vulva, vagina, cervix, anus, larynx, and oropharynx

Front 69

What cancers are associated with HHV-8

Back 69

Kaposi's sarcoma

Front 70

What cancer is associated with HBV and what is the mechanism?

Back 70

Hepatocellular carcinoma. HBV inactivates P53 suppressor gene

Front 71

What cancers are associated with H. pylori?

Back 71

H. pylori can cause gastric lymphoma and gastric carcinoma

Front 72

What cancers are associated with HTLV-1 (Human T Lymphotropic Virus)?

Back 72

T-cell leukemia and lymphoma