S1B6 - Cell accumulation & aging Flashcards
Primary amyloidosis occurs from amyloid deposition in tissues of which embryological origin?
Amyloid deposition in tissues of mesodermal origin, such as heart, muscle, and tongue is characteristic.
Which is characterized by episodic fever and polyserositis?
A) Portuguese type of polyneuropathy
B) Alzheimer disease
C) Familial Mediterranean fever
D) Medullary carcinoma of the thyroid
E) Diabetes mellitus
F) Senile amyloidosis
G) Dialysis-associated amyloidosis
Familial Mediterranean fever
What is the name of the important heat shock chaperone protein induced by stress which marks abnormal proteins for degradation?
ubiquitin
In which is it postulated that amylin interferes with insulin sensing by beta cells?
A) Portuguese type of polyneuropathy
B) Alzheimer disease
C) Familial Mediterranean fever
D) Medullary carcinoma of the thyroid
E) Diabetes mellitus
F) Senile amyloidosis
G) Dialysis-associated amyloidosis
Diabetes mellitus
What pigment can be positively identified by its staining reaction (blue color) with Prussian blue dye?
Hemosiderin
Protein folding disorders involve failure of protein structural stabilization or degradation by specialized proteins known as ______________.
chaperones
Reabsorption droplets in proximal renal tubules are a sign of what kind of accumulation?
protein accumulation
Is amyloid characteristically intracellular or extracellular in distribution?
Amyloid is characteristically extracellular in distribution, most often appearing as accumulations proximate to basement membranes.
Which is characterized by severe peripheral nerve involvement caused by amyloid deposits?
A) Portuguese type of polyneuropathy
B) Alzheimer disease
C) Familial Mediterranean fever
D) Medullary carcinoma of the thyroid
E) Diabetes mellitus
F) Senile amyloidosis
G) Dialysis-associated amyloidosis
Portuguese type of polyneuropathy
What is “sago spleen,” an appearance reminiscent of tapioca-like granules, characteristic of?
Perifollicular involvement in the spleen with secondary amyloidosis.
What pigment exists normally in small amounts as physiologic iron stores within tissue macrophages of the bone marrow, liver, and spleen?
Hemosiderin
What are the two known pathogenic mechanisms of protein accumulation?
- Abnormal protein aggregation, which is characteristic of amyloidosis; a number of neurodegenerative diseases, such as Alzheimer disease, Huntington disease, and Parkinson disease; and perhaps prion diseases, such as “mad cow” disease
- Abnormal protein transport and secretion, which is characteristic of cystic fibrosis and α1antitrypsin deficiency.
What pigment accumulates and stains the blood, sclerae, mucosae, and internal organs, producing a yellowish discoloration called jaundice?
bilirubin
Which is caused by hypercalcemia?
A) Metastatic calcification
B) Dystrophic calcification
Metastatic calcification
Describe the structure of an amyloid.
Atherosclerosis, xanthomas, and foamy macrophages secondary to inflammation and necrosis result from pathologic accumulations of what type of molecule?
cholesterol
All normal cells have a limited capacity for replication, and after a fixed number of divisions cells become arrested in a terminally nondividing state. What is this called?
Waldenström macroglobulinemia and multiple myeloma are plasma cell disorders that are frequently associated with which amyloidosis:
A) Primary amyloidosis
B) Secondary amyloidosis
C) Portuguese type of polyneuropathy
D) Alzheimer disease
E) Senile amyloidosis
Primary amyloidosis
What leads to increased serum amyloid-associated protein (SAA)?
Chronic tissue destruction leads to increased SAA.
What are some examples of accumulations of exogenous pigments?
The gene that codes for the protein precursors of A4 amyloid has been localized to which chromosome?
chromosome 21
What pigment commonly accumulates in elderly patients, in whom the pigment is found most often within hepatocytes and at the poles of nuclei of myocardial cells?
Lipofuscin
What pigment is decreased in albinism and vitiligo?
melanin
What organs are typically involved with secondary amyloidosis (reactive systemic amyloidosis)?
Usually, involvement of parenchymatous organs, especially the kidneys (nephrotic syndrome is very common), liver, adrenals, pancreas, lymph nodes, and spleen is seen.
What is cellular aging the result of?
Cellular aging is the result of a progressive decline in cellular function and viability caused by genetic abnormalities and the accumulation of cellular and molecular damage due to the effects of exposure to exogenous influences.
What cells synthesize melanin? Where in those cells are they synthesized?
Which is characterized by deposits of an amyloid protein referred to as A4 amyloid, or amyloid b-protein, which differs from AL, AA, and transthyretin-derived amyloid?
A) Portuguese type of polyneuropathy
B) Alzheimer disease
C) Familial Mediterranean fever
D) Medullary carcinoma of the thyroid
E) Diabetes mellitus
F) Senile amyloidosis
G) Dialysis-associated amyloidosis
Alzheimer disease
What is the classic appearance of accumulated cholesterol?
Classic appearance of accumulated cholesterol:
- “Cholesterol clefts”
- “Foam cells”
Does amyloid have an eosinophilic or basophilic appearance in routine hematoxylin and eosin sections?
It has an amorphous eosinophilic appearance in routine hematoxylin and eosin sections.
What causes increased melanin pigmentation?
Where in the body is fatty change (fatty metamorphosis and steatosis) most frequently found?
Fatty change is characterized by the accumulation of intracellular parenchymal triglycerides and is observed most frequently in the liver, heart, and kidney.
What does telomerase use to add nucleotides to the ends of chromosomes?
Telomerase is a specialized RNA-protein complex that uses its own RNA as a template for adding nucleotides to the ends of chromosomes.
What are some common causes of hypercalcemia that can lead to metastatic calcification?
What is amyloid characteristically stained by? What is the appearance of amyloid stained with this dye?
Amyloid is characteristically stained by Congo red dye, demonstrating apple green birefringence when viewed under polarized light and confirming the suspected presence of amyloid.
What’s the difference between hemosiderosis and hemochromatosis?
What pigment is a catabolic product of the heme moiety of hemoglobin and, to a minor extent, myoglobin?
bilirubin
What causes secondary amyloidosis?
Secondary amyloidosis (reactive systemic amyloidosis):
Deposition of fibrils consisting of the amyloid protein called AA protein, which is formed from a precursor, serum amyloid-associated protein (SAA) is characteristic.
What pigment is sometimes referred to as “wear-and-tear” pigment?
Lipofuscin
Which is characterized by amyloid deposits in the joints of patients who have undergone hemodialysis for several years?
A) Portuguese type of polyneuropathy
B) Alzheimer disease
C) Familial Mediterranean fever
D) Medullary carcinoma of the thyroid
E) Diabetes mellitus
F) Senile amyloidosis
G) Dialysis-associated amyloidosis
Dialysis-associated amyloidosis
What pigment is involved with hemosiderosis and hemochromatosis?
Hemosiderin
Why are the most common hematologic malignancies are diseases of the aged?
Which is an autosomal recessive disorder occurring in persons of Eastern Mediterranean origin?
A) Portuguese type of polyneuropathy
B) Alzheimer disease
C) Familial Mediterranean fever
D) Medullary carcinoma of the thyroid
E) Diabetes mellitus
F) Senile amyloidosis
G) Dialysis-associated amyloidosis
Familial Mediterranean fever
X-ray diffraction demonstrates what structural feature of amyloids?
β-pleated sheet configuration
Which has a distribution and type of amyloid similar to that of secondary amyloidosis (AA amyloid)?
A) Portuguese type of polyneuropathy
B) Alzheimer disease
C) Familial Mediterranean fever
D) Medullary carcinoma of the thyroid
E) Diabetes mellitus
F) Senile amyloidosis
G) Dialysis-associated amyloidosis
Familial Mediterranean fever
Which is associated with an amyloid derived from a protein known as transthyretin (a serum protein that transports thyroxine and retinol).
A) Portuguese type of polyneuropathy
B) Alzheimer disease
C) Familial Mediterranean fever
D) Medullary carcinoma of the thyroid
E) Diabetes mellitus
F) Senile amyloidosis
G) Dialysis-associated amyloidosis
Portuguese type of polyneuropathy
What is the combination of lipofuscin accumulation and atrophy of organs is referred to as?
brown atrophy
What is the cause of fatty change (fatty metamorphosis and steatosis)?
Imbalance among the uptake, utilization, and secretion of fat is the cause of fatty change.
Which type of calcification is defined as calcification in previously damaged tissue, such as areas of old trauma, tuberculosis lesions, scarred heart valves, and atherosclerotic lesions?
A) Metastatic
B) Dystrophic
Which, when occurs in the heart, the amyloid protein is derived from transthyretin?
A) Portuguese type of polyneuropathy
B) Alzheimer disease
C) Familial Mediterranean fever
D) Medullary carcinoma of the thyroid
E) Diabetes mellitus
F) Senile amyloidosis
G) Dialysis-associated amyloidosis
Senile amyloidosis
What is the characteristic appearance of hyaline change in hematoxylin and eosin sections?
(homogeneous, glassy, and eosinophilic)
Russell Bodies in alcoholic liver disease are a sign of what kind of accumulation?
protein accumulation
Which is characterized by minor deposits of amyloid found at autopsy in the very elderly?
A) Portuguese type of polyneuropathy
B) Alzheimer disease
C) Familial Mediterranean fever
D) Medullary carcinoma of the thyroid
E) Diabetes mellitus
F) Senile amyloidosis
G) Dialysis-associated amyloidosis
Senile amyloidosis
Which has amyloid thought to be derived from either insulin or glucagon and is referred to as amylin or, alternatively, islet amyloid polypeptide?
A) Portuguese type of polyneuropathy
B) Alzheimer disease
C) Familial Mediterranean fever
D) Medullary carcinoma of the thyroid
E) Diabetes mellitus
F) Senile amyloidosis
G) Dialysis-associated amyloidosis
Diabetes mellitus
What pigment is formed from tyrosine by the action of tyrosinase?
melanin
Which is characterized by prominent amyloid deposits within the tumor, apparently derived from calcitonin?
A) Portuguese type of polyneuropathy
B) Alzheimer disease
C) Familial Mediterranean fever
D) Medullary carcinoma of the thyroid
E) Diabetes mellitus
F) Senile amyloidosis
G) Dialysis-associated amyloidosis
Medullary carcinoma of the thyroid
Secondary amyloidosis is characteristically a complication of what diseases?
Secondary amyloidosis characteristically is a complication of chronic inflammatory disease, such as rheumatoid arthritis, tuberculosis, osteomyelitis, syphilis, or leprosy. It also may complicate noninflammatory disorders, such as renal cell carcinoma and Hodgkin disease.
What pigment is an iron-containing pigment consists of aggregates of ferritin?
hemosiderin
What allows immortalized cancer cells to proliferate indefinitely?
Conversely, in immortalized cancer cells, telomerase is usually reactivated and telomere length is stabilized, allowing the cells to proliferate indefinitely.
What pigment appears in tissues as golden brown amorphous aggregates?
Hemosiderin
Compare telomerase activity of germ cells, stem cells, and somatic tissues.
Telomerase activity is expressed in germ cells and is present at low levels in stem cells, but it is absent in most somatic tissues
What is the pathology word for a group of disorders is characterized by the deposition of a proteinaceous material with certain physicochemical features?
AMYLOIDOSIS
What enzyme mediates the addition of nucleotides to maintain telomere length?
telomerase
What is the cause of primary amyloidosis?
The cause is deposition of amyloid fibrils derived from immunoglobulin light chains, referred to as AL (amyloid light chain) protein.
The amyloid of which is derived from β-microglobulin?
A) Portuguese type of polyneuropathy
B) Alzheimer disease
C) Familial Mediterranean fever
D) Medullary carcinoma of the thyroid
E) Diabetes mellitus
F) Senile amyloidosis
G) Dialysis-associated amyloidosis
Dialysis-associated amyloidosis
What yellowish, fat-soluble pigment is an end product of membrane lipid peroxidation?
Lipofuscin
Which is characterized by deposits of amyloid in islet cells in the insulin-resistant adult-onset form (type 2)?
A) Portuguese type of polyneuropathy
B) Alzheimer disease
C) Familial Mediterranean fever
D) Medullary carcinoma of the thyroid
E) Diabetes mellitus
F) Senile amyloidosis
G) Dialysis-associated amyloidosis
Diabetes mellitus
