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55 Cards in this Set

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3 categories of intracellular accumulations:
1. Normal substances in excess
2. Abnormal substances
3. Pigments
What are 3 mechanisms of intracellular accumulations?
1. Inadequate rate of metabolism
2. Genetic or acquired defect
3. Exogenous substance can't be metabolized
What is Steatosis?
The abnormal accumulation of triglycerides in a cell
What are 4 causes of Steatosis?
-Toxins (alcohol)
-Protein malnutrition
-Diabetes
-Obesity
What is the mechanism that results in steatosis?
A defect in uptake, catabolism, or secretion of fatty acids
In what tissue does steatosis most often occur?
The liver (but also can be heart and muscle)
Where do cholesterol accumulations occur in cells?
In intracellular vacuoles
Where does cholesterol lipid accumulate in Atherosclerosis?
-Smooth muscle cells
-Foamy macrophages
What is Xanthoma?
The accumulation of cholesterol in macrophages in hyperlipidemic diseases.
Why does cholesterol accumulate in cells during inflammation and necrosis?
Because it is phagocytosied from the plasma membrane.
What is Cholesterolosis?
The condition where macrophages in the gallbladder are filled with cholesterol.
What is the characteristic finding of cholesterolosis?
Strawberry gallbladder
What is the lysosomal storage disorder in which cholesterol trafficking is defective?
Niemann pick disease, type C
What are 2 situations in which Proteins can accumulate within cells?
-Proteinuria
-Defects in protein folding
What morphologic feature results from proteinuria?
Reabsorption droplets in proximal renal tubules
What are 5 protein folding defect diseases where protein accumulates in cells?
1. a-1-antitrypsin deficiency
2. Cystic fibrosis
3. Familial hypercholesterolemia
4. Neurodegenerative diseases
5. Amyloidosis
Why does alpha 1 antitrypsin deficiency cause protein aggregation?
Because of slow folding proteins aggregate in the ER
What are Russell bodies?
Eosinophilic inclusions found in immne cells when secretory proteins are produced excessively.
What is amyloidosis?
The aggregation of proteins
What exactly is a Hyaline change?
A homogenous, glassy, pink appearance.
What are 2 accumulations that can cause such a hyaline change?
-Intracellular protein accumulations
-Collagenous fibrous tissue
What does glycogen accumulation result from?
Abnormal glucose or glycogen metabolism
What is the histological appearance of glycogen accumulations?
Clear vacuoles
What are 2 diseases that glycogen accumulations are associated with?
-Diabetes mellitus
-Glycogen storage diseases
What disease correlates wtih excess glycogen in the myocardium? What is the abnormal enzyme?
Pompe disease
-Lysosomal glucosidase
What is von Gierke disease? What organ is associated?
G6P deficiency - accumulations in the liver
What is muscle phosphorylase deficiency?
McArdle
What are 2 exogenous pigments that can be found accumulated in cells?
-Carbon
-Tattoo ink
What are 4 endogenous pigments?
-Lipofuscin
-Melanin
-Hemosiderin
-Bilirubin
What is lipofuscin?
A polymer of lipid and protein
What is lipofuscin a sign of?
Free radical injury
What are the morphologic characteristics of lipofuscin?
-Yellow brown
-Finely granular
-Perinuclear
What is carbon in the lung called?
Lung anthracosis
What will melanin pigment be seen in?
Melanoma
What is hemosiderin?
Iron
What is localized hemosiderin a common finding in?
Bruises - contusion
What are the 4 stages of pigmenting seen in bruises, and what color are they?
1. Hemorrhage - red/blue
2. Biliverdin - green blue
3. Bilirubin
4. Hemosiderin - golden/yellow
When can systemic iron overload be seen?
1. Transfulsional or 2ndary hemosiderosis
2. Hereditary hemachromatosis
What will hemosiderin be found accumulated in?
Hemosiderin-laden macrophages
What is hemachromatosus?
Increased iron uptake from the diet
What are the 2 types of pathologic calcifications?
1. Dystrophic
2. Metastatic
Where does dystrophic calcification occur?
Locally in dying tissues
What will serum levels of Ca be like in dystrophic calcification?
Normal
What are 2 diseases where dystrophic calcification occurs?
-Atherosclerosis
-Calcified heart valves
What are 2 findings that may occur in dystrophic calcification?
-Heterotopic bone
-Psammoma bodies
What are Psammoma bodies?
Progressively acquired layers of calcium
What is metastatic calcification?
The deposition of calcium in otherwise normal tissues.
What is metastatic calcification almost always associated with?
Calcium metabolism disturbances
What are 4 common causes of metastatic calcification?
1. HyperPTH
2. Bone destruction (mx myeloma)
3. Vit D disorders
4. Renal failure
So dystrophic is due to
cellular necrosis
And metastatic is due to
metabolic disturbances
What are 5 common sites for metastatic calcification?
-GI mucosa
-Kidneys
-Lungs
-Systemic arteries
-Pulmonary veins
What do the sites of metastatic calcification have in common?
alkaline
What are 3 things that accumulate due to normal cellular aging?
-Lipofuscin
-Glycation end products
-Abnormally folded proteins
What are 3 causes of cellular aging?
1. Replicative senescence
2. Influential genes
3. Accmulation of metabolic and genetic damage