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Pathomorphology > INTRACELLULAR ACCUMULATIONS (PARENCHYMAL DEGENERATIONS OR DYSTROPHIES) > Flashcards

INTRACELLULAR ACCUMULATIONS (PARENCHYMAL DEGENERATIONS OR DYSTROPHIES) Flashcards

1
Q

What three categories do stockpiled substances fall into?

A
  1. A normal cellular constituent accumulated in excess, such as water, lipid, protein, and carbohydrates.
  2. An abnormal substance such as mineral, or a product of abnormal metabolism.
  3. A pigment or an infectious product.
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2
Q

In what types of cells do parenchymal degenerations occur? Give examples

A

Functional cells such as cells of the liver, kidneys and myocardium

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3
Q

How are parenchymal degenerations characterised?

A

An accumulation in the cells of proteins, lipids and fats

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4
Q

What features accompany intracellular accumulations?

A

A decrease in the function of enzymic systems and a change in the structure of cells

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5
Q

What are the most frequent causes of parenchymal degenerations?

A

Hypoxia, intoxication and enzymopathy

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6
Q

What are enzymopathies?

A

Genetically determined diseases at which is observed an inconsistency of enzymic systems in cells

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7
Q

What is the result of enzymopathies?

A

Accumulation in the cells of the products of metabolism known as storage diseases

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8
Q

What are the four kinds of intracellular accumulations of proteins?

A

A granular degeneration (dystrophy)
Hyaline-drop degeneration (dystrophy)
Hydropic (cloudy, vacuolar, balloon) degeneration
Keratoid (horney) degeneration

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9
Q

In granular degeneration what is the macroscopic appearance of the organ?

A

Muddy or dim swelling

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10
Q

In granular degeneration what is the macroscopic appearance of a cut section?

A

Dim and swollen

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11
Q

In granular degeneration what is the microscopic appearance of the cells?

A

Electrodense granules in the cytoplasm of cells

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12
Q

How is hyaline drop degeneration characterised?

A

Aggregation of small proteins granules into cytoplasm of cells

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13
Q

Is hyaline drop dystrophy determined macroscopically?

A

No

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14
Q

In what organs does hyaline drop degeneration occur?

A

Kidneys, heart and liver

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15
Q

In hyaline drop degeneration what can be seen in the cytoplasm of plasma cells?

A

The cytoplasm of plasma cells shows pink hyaline inclusions called Russell’s bodies representing synthesised immunoglobulins

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16
Q

In hyaline drop degeneration what can be seen in the cytoplasm of hepatic cells?

A

The cytoplasm of hepatocytes shows eosinophilic globular deposits of a mutant protein

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17
Q

What are Mallory’s bodies?

A

Mallory’s body or alcoholic hyaline in the hepatocytes is intracellular accumulation of intermediate filaments of cytokeratin

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18
Q

What is the usual outcome of hyaline drop degeneration?

A

The outcome is negative. The focal or total coagulative necrosis develops.

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19
Q

How is hydropic (cloudy, balloon, vacuolar) degeneration characterised?

A

The common causes include bacterial toxins, chemicals, poisons, burns, and high fever

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20
Q

What happens to affected organs in hydropic degeneration?

A

Enlargement

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21
Q

How does the cut surface look in hydropic degeneration?

A

Bulges outwards and is slightly opaque

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22
Q

What is seen under the microscope in hydropic degeneration?

A 
Microscopically: the cells are swollen and the microvasculature compressed. Small clear vacuoles are seen in the cells. These vacuoles represent distended cisternas of the endoplasmic reticulum. Ultrastructural changes in hydropic swelling include the following:
•	Dilation of endoplasmic reticulum.
•	Mitochondrial swelling.
•	Blebs on the plasma membrane.
•	Loss of fibrillanty of nucleolus.
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23
Q

What is the outcome of hydropic degeneration?

A

The outcome is negative, because the focal or total colliquative cellular necrosis develops.

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24
Q

How is keratoid (horney) degeneration characterised?

A

Increased production of keratin substance

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25
Q

Where can excess keratin production be in keratoid degeneration?

A

This process may be local and general. The intracellular keratin may be located in epidermis of skin, keratinic squamous epithelial cells, cervix, and esophagus.

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26
Q

What is leucoplakia?

A

Leucoplakia means hyperkeratosis in mucosa.

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27
Q

What is the most severe complication of leucoplakia?

A

Leucoplakia may lead to malignization. For example: Squamous cell carcinoma with keratinization

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28
Q

Describe the appearance of the keratinised areas within squamous cell carcinomas

A

These cell’s complexes here and there look like rose color homogenous found forms (“canceromatous perls”).

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29
Q

What is the main cause of fatty intracellular degenerations?

A

The main cause of fatty degeneration is hypoxia

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30
Q

What might cause the hypoxia which leads to intracellular fatty degenerations?

A
  1. Excess alcohol consumption (most commonly).
  2. Chronic cardiovascular and chronic pulmonary insufficiency.
  3. Cachexia, avitaminosis.
  4. Infections (e.g. diphtheria, tuberculosis).
  5. Late period of pregnancy.
  6. Starvation.
  7. Malnutrition.
  8. Hepatotoxins (e. g. carbon tetrachloride, chloroform).
  9. Certain drugs (e. g. administration of estrogen, steroids, tetracycline).
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31
Q

What are the mechanisms by which alcoholism leads to intracellular fatty accumulations?

A

In the case of cell injury by chronic alcoholism, many factors are implicated with increased lipolysis, increased free fatty acid synthesis, decreased tryglyceride utilisation, decreased fatty acid oxidation to ketone bodies, and block in lipoprotein excretion.

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32
Q

How can fat in tissues be demonstrated?

A

Fat in the tissue can be demonstrated by frozen section followed by fat stains such as Sudan 3 (red color), oil red O and osmic acid.

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33
Q

What is the macroscopic appearance in fatty degeneration of the liver?

A

Macroscopically the fatty liver is enlarged with rounded margins.

34
Q

What does the cut surface of a liver look like if there is fatty degeneration?

A

The cut surface bulges slightly and is pale-yellow and is greasy to touch. It is called “goose liver”.

35
Q

Describe the microscopic appearance of the liver if intracellular fatty accumulations are present

A
  • Microscopically: there are numerous lipid vacuoles in the cytoplasm of hepatocytes. The vacuoles are initially small (microvesicular), but with progression of the process, the vacuoles become larger pushing the nucleus to the periphery of the cells (macrovesicular).
  • At times, the hepatocytes laden with large lipid vacuoles may rupture and lipid vacuoles coalesce to form fatty cysts. Infrequently, lipogranulomas may appear.
36
Q

What is the name for the appearance of parenchymal fatty degenerations of the heart?

A

Tiger’s heart

37
Q

Describe the macroscopic appearance of the heart in parenchymal fatty degeneration

A

Macroscopically the heart is enlarged, the chambers are stretched, flabby.

38
Q

Describe the microscopic appearance of the heart in parenchymal fatty degeneration

A
  • Microscopically we can see dust-like fatty vacuoles in the cardiomyocytes.
  • It is observed in the papillary muscles and trabecules of the ventricles in the form of bands (surrounding the veins).
39
Q

What is the appearance of the kidney in parenchymal fatty degeneration?

A

The kidneys look like “large white kidney”. They are enlarged, flabby.

40
Q

What is the microscopic appearance of the kidney in parenchymal fatty degeneration?

A

The cortical substance is grey with yellow drops.

41
Q

What is the outcome of fatty parenchymal ddegenerations?

A

Outcomes of fatty degenerations are seldom reversible. Necrosis or sclerosis may develop.

42
Q

What groups are carbohydrates divided into?

A
  1. Polysaccharides (glycogen).
  2. Mucopolysaccharides.
  3. Glycoproteides (mucin, mucoid).
43
Q

What staining can be used to confirm the presence of glycogen in cells?

A

Best’s carmine and PAS (periodic acid-Schiff) staining may be employed to confirm the presence of glycogen in cells.

44
Q

What colour are polysaccharides (glycogen) and mucopolysaccharides stained with Best’s carmine and PAS?

A

Polysaccharides and mucopolysaccarides are stained dark pink or red.

45
Q

What stain is used for glycoproteides (mucin,mucoid)?

A

Haile

46
Q

What colour do glycoproteides stain with Haile?

A

Blue

47
Q

What abnormality usually leads to accumulation of intracellular glycogen?

A

Abnormality of either glucose or glycogen metabolism

48
Q

What is the microscopic appearance of tissues with parenchymal glycogen accumulation?

A

Appearance of glycogen masses as clear vacuoles within the cytoplasm developed with special stain – PAS-reaction

49
Q

What is the prime example of an abnormality that causes intracellular glycogen accumulation?

A

Diabetes mellitus

50
Q

What is observed microscopically in the kidneys in patients with diabetes mellitus?

A

Red colour granules of glycogen can be found with large magnification in the epithelial cells of Henley’s loops and in the lumen of kidney’s canals

51
Q

What is a complication of accumulations of glycogen?

A

Amount of glycogen in the tissues reduces sharply (e.g. in the liver) which causes its fat infiltration (fatty liver degeneration).

52
Q

What accumulates in mucoid change?

A

Mucin

53
Q

What stains are used to stain epithelial and connective tissue mucin and what colour is produced?

A

Epithelial mucin is stained positively with periodic acid-Shiff (PAS), while connective tissue mucin does not but is stained positively with colloidal iron. Both are, however, stained by alcian blue.

54
Q

What is epithelial mucin associated with?

A
  • Catarrhal inflammation of mucous membrane (e. g. of respiratory tract, alimentary tract, uterus).
  • Obstruction of duct leading to mucocele in the oral cavity, chronic appendicitis and gall bladder.
  • Cystic fibrosis of the pancreas or mucoviscidosis.
  • Mucin-secreting tumors (e. g. of ovary, stomach, large bowel etc.).
55
Q

What are storage diseases?

A

There are a lot of diseases, which are due to hereditary factors and connected with metabolism disturbance. These diseases are called storage diseases or enzymopathy.

56
Q

Give two general comments that can be made about storage diseases

A
  • All the storage diseases occur as a result of autosomal recessive, or sex-(X-) linked recessive genetic transmission.
  • Most of the storage diseases are lysosomal storage diseases. Out of the glycogen storage diseases, only type II (Pompe’s disease) is due to lysosomal enzyme deficiency
57
Q

What are the three types of storage diseases?

A
  • Proteinoses
  • Lipidosis
  • Glucogenoses
58
Q

What are the most frequent lipidoses?

A

Gaucher’s disease, Niemann-Pick disease

59
Q

What is Gaucher’s disease?

A

This is an autosomal recessive disorder in which there is deficiency of lysosomal enzyme, glucocerebrosidase, which normally cleaves glucose from ceramide.

60
Q

What is the consequence of glucocerebrosidase deficiency in Gaucher’s disease?

A

This results in lysosomal accumulation of glucocerebroside (ceramide-glucose) in phagocytes of the body and sometimes in the neurons.

61
Q

What are the main sources of glucocerebroside in the phagocytes and in the neurons?

A

The main sources of glucocerebroside in phagocytic cells of the body and sometimes in the neurons are the membrane glycolipids of old leukocytes and erythrocytes, while the deposits in the neurons consist of gangliosides.

62
Q

Describe the three clinical types of Gaucher’s disease

A
  1. Type 1 or classic form is the adult form of the disease in which there is storage of glycocerebrosides in the phagocytes of the body, principally involving the spleen, liver, bone marrow and lymph nodes. This is the most common type comprising 80% of all cases of Gaucher’s disease.
  2. Type II is the infantile form in which there is progressive involvement of the central nervous system.
  3. Type III is the juvenile form of the disease having features in between type I and type II, i.e. they have systemic involvement like in type I and progressive involvement of the central nervous system (CNS) as in type II.
63
Q

Give the macroscopic features of Gaucher’s disease

A

In addition to involvement of different organs and systems (splenomegaly, hepatomegaly, lymphadenopathy, bone marrow and cerebral involvement), a few other features include bone pains and pathologic fractures.

64
Q

Give the haematological features of Gaucher’s disease

A

Pancytopenia, or thrombocytopenia secondary to hypersplenism,

65
Q

Give the microscopic features of Gaucher’s disease

A

Microscopically large number of characteristically distended and enlarged macrophages called Gaucher cells which are found in the spleen, liver, bone marrow and lymph nodes, and in the case of neuronal involvement, in the Virchow-Robin space. The cytoplasm of these cells is abundant, granular and fibrillar resembling crumpled tissue paper. They have mostly a single nucleus but occasionally may have two or three nuclei. These cells often show erythrophagocytosis and are rich in acid phosphatase.

66
Q

What is Niemann-Pick disease?

A

This is also an autosomal recessive disorder characterized by accumulation of sphingomyelin and cholesterol.

67
Q

What is the cause of accumulations in Niemann-Pick disease?

A

• Majority of the cases (about 80%) have deficiency of sphingomyelinase, which is required for cleavage of sphingomyelin, while a few cases probably result from deficiency of an activator protein.

68
Q

When does Niemann-Pick disease present?

A

• The condition presents in infancy

69
Q

How does Niemann-Pick disease present macroscopically?

A
  • The condition is characterized by hepatosplenomegaly, lymphadenopathy and physical and mental underdevelopment.
  • About a quarter of patients present with familial amaurotic idiocy with characteristic cherry-red spots in the macula of the retina.
70
Q

Where is sphingomyelin stored in Niemann-Pick disease?

A

The storage of sphingomyelin and cholesterol occurs within the lysosomes, particularly in the cells of mononuclear phagocyte system.

71
Q

How are the phagocytes different in Niemann-Pick disease than in Gaucher’s disease?

A
  • The cells of Niemann-Pick disease are somewhat smaller than Gaucher cells and their cytoplasm is not wrinkled but is instead foamy and vacuolated which stains positively with fat stains.
  • These cells are located in the spleen, liver, lymph nodes, bone marrow, lungs, intestine and brain.
72
Q

What are the most common glycogen storage dieases?

A

The most frequent glycogen storage diseases or glycogenosis are Pompe’s disease, Mc Ardle’s disease and Gierke disease. There is defective metabolism of glycogen due to genetic disorders.

73
Q

What is Pompe’s disease?

A

This is also an autosomal recessive disorder due to deficiency of a lysosomal enzyme, acid mahase.

74
Q

What is the consequence of acid mahase deficiency in Pompe’s disease?

A

Its deficiency results in accumulation of glycogen in many tissues, most often in the heart and skeletal muscles leading to cardiomegaly and hypotonia.

75
Q

What is McArdle’s disease?

A

The condition occurs due to deficiency of muscle phosphorylase resulting in accumulation of glycogen in the muscle (deficiency of liver phosphorylase)

76
Q

What ages are usually affected by McArdle’s diease?

A

The disease is common in 2nd to 4th decades of life

77
Q

How is McArdle’s disease characterised?

A

Painful muscle cramps, especially after exercise, and detection of myoglobinuria in half the cases.

78
Q

What is Gierke disease?

A

This condition is inherited as an autosomal recessive disorder due to deficiency of enzyme, glucose-6-phosphatase

79
Q

What are the consequences of the deficiency of enzyme glucose-6-phosphatase in Gierke’s disease?

A

In the absence of glucose-6-phosphatase, excess of normal type of glycogen accumulates in the liver and also results in hypoglycemia due to reduced formation of free glucose from glycogen. As results, fat is metabolized for energy requirement leading to hyperlipoproteinemia and ketosis. Other changes due to deranged glucose metabolism are hyperuricemia.

80
Q

What are the macroscopic features of Gierke disease?

A

The disease manifests clinically in infancy with failure to thrive and stunted growth. Most prominent feature is enormous hepatomegaly. The kidneys are also enlarged. Other features include gout, skin xanthomas and bleeding tendencies due to platelet dysfunction.

81
Q

What are the microscopic features of Gierke disease?

A

Intracytoplasmic and intranuclear glycogen in hepatocytes. Intracytoplasmic glycogen in tubular epithelial cells.

82
Q

Is the outcome of storage disease favourable of unfavourable?

A

The outcome of storage diseases is unfavorable because of insufficienty of the respective organ.

Pathomorphology (3 decks)

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