PIGMENTS Flashcards
What are cell pigments?
Accumulation of color substances within or outside the cell
What are the 2 types of Pigments?
1) Exogenous Pigments: Produced outside the body
2) Endogenous Pigments: Produced inside the body. Some are normal and some pathological in nature
What are the 2 EXOGENOUS PIGMENTS?
1) Carbon or Coal
2) Tattooing
What is the most common pathological pigment?
Carbon or coal
Anthracosis aka
Coal Workers Pneumoconiosis
What is Anthracosis aka Coal Worker’s Pneumoconiosis?
Accumulation of coal in the lungs characteristic of mine workers. Coal dust coats the bronchioles and gets in the lymph nodes.
What happens during Anthracosis?
Lung tissue is replaced by connective tissue as an attempt to get rid of the coal from the lung - inflammation, fibrosis, and even necrosis occurs
What does the term Pneumoconiosis mean?
It is a general term for lung diseases caused by scar tissue formation after inhaling some particle
Worker’s Pneumoconiosis is the ______
Severe state of a milder initial disease anthracosis
What happens from tattooing?
Ink goes into dermatocytes, and usually does not involve pathology
What can happen from tattooing?
An allergic reaction can happen causing painful blisters, and the only way to get rid of it is by burning
What are the ENDOGENOUS PIGMENTS?
1) Lipofuscin (aka lipochrome)
2) Melanin
3) Homogentisic Acid
4) Hemosiderin
5) Accumulation of Copper
What is Lipofuscin aka
Lipochrome
What is Lipofuscin?
Yellowish-Brown pigment that usually develops as a result of atrophy or overloading of the tissue
What is Lipofuscin known as?
Wear and Tear pigment aka brown atrophy, and it also may develop from aging.
What does Lipfuscin represent?
Complexes of protein and lipid that derived from free radical peroxidation of polyunsaturated lipids of sub cellular membranes
Is the Lipofuscin pigment dangerous?
No, it is normal and is referred to as brown atrophy
Where is Lipfuscin found?
In the heart, brain, and liver. Its presence indicates that there was previous injury or aging
What is Melanin?
A black pigment made naturally from Tyrosine in melanocytes
What does Melanin do?
It gives the skin its color and protects from UV radiation of the sun
What is Homogentisic acid?
PATHOLOGICAL black pigment.
In what scenario would you find the accumulation of Homogentisis acid?
It is a normal metabolic intermediate, but in the pathological condition alkoptonuria it accumulates in the skin and develops as a black pigment
What is also black with patients who have alkoptonuria?
Urine
What is Hemosiderin?
It is an iron storage complex found within cells in the form of Ferritin molecules
Where is Hemosiderin mostly found?
In macrophages: When a blood vessel ruptures and RBC’s die they release Hb into the extracellular space - Macrophages will engulf the Hb to decrease it producing Homosiderin as a product
Where may Excessive accumulation of homosiderin be seen?
It may be seen in increased mononuclear macrophages in tissues like liver, kidneys, lymph nodes, bone marrow, lung, and spleen
What is homosiderin seen in those organs due to?
It is due to local or systemic excess of iron: Excessive destruction of RBC’s, excessive intake of iron or decreased iron utilization
What are the 2 subcategories associated with Hemosiderin?
Hemosiderosis and Hemochromatosis
What is Hemosiderosis?
Deposition of hemosiderin in many organs and tissues usually after hemorrhage (abundant during bruising)
Hemosiderosis’s characteristics:
May be local, affecting one organ or systemic, affecting several organs. Usually not dangerous
What are normal homosiderin levels?
2-5 grams
What is Homosiderosis?
A low grade accumulation of homosiderin (>5 grams)
What is a common cause of Hemosiderosis?
Hemolytic anemia (sickle cell) - causes a black eye
Does Hemosiderosis damage organs?
NO
What is the key feature of Hemochromatosis?
Dangerous because it is associated with extreme iron accumulation
What is the normal amount of iron in the body to be considered safe?
6 grams, (Hemochromatosis can be 50 grams of iron)
Hemochromatosis can be what type of disease?
Genetic or Acquired
How can you acquire or genetically be predisposition to Hemochromatosis ?
Hemolytic anemia, drinking alcohol from bottles made with iron, cardiac failure, diabetes mellitus, and it interferes with DNA
Hemochromatosis more prevalent in?
MALES
What are the symptoms of Hemochromatosis?
Abdominal pain, joint pain, hepatomegaly, and hyper pigmentation of the skin
What are the 2 types of Hemochromatosis?
1) Primary or Hereditary hemochromatosis
2) Secondary iron Overload
What is the definition of primary or hereditary hemochromatosis?
Genetic disease only seen in boys
What happens due to Primary or hereditary hemochromatosis?
- Liver Fibrosis/cirrhosis (HIGH chance of LIVER CANCER)
- Pancreas damage
- DNA damage
What is “Secondary Iron Overload? (associated with Hemochromatosis) “
Acquired systemic overload of iron due to over-destruction of RBC’s in a rejection of blood transfusion, iron supplementation (poisoning)
What is an example of Secondary Iron Overload (associated with Hemochromatosis)?
Bantu Siderosis: Very rare disease in Africa where alcohol is stored in iron containers. Iron passes to alcohol and it is drunk
What is the most vulnerable organ?
Liver, and it can lead to liver cancer (hepatoma)
What are manifestations of Hemochromatosis?
Skin pigmentation especially in sun exposed areas, abdominal pain, arrhythmia.
What other affects do Hemochromatosis have?
It affects the pancreas especially in Diabetes Mellitus. Liver fibrosis, cardiac failure, and diabetes mellitus
What disease is the accumulation of Copper?
Wilson’s disease
What is the aka of Wilson’s disease?
Hepatolenticular Degeneration
What is Wilson’s disease aka Hepatolenticular Degeneration?
A genetic disease that leads to copper accumulation in the body due to excess absorption.
What normally happens to copper in the body?
It is absorbed and goes to the liver, and binds with Alpha 2 globulin*
When copper binds with Alpha 2 globulin what does it form?
Ceruloplasmin (95% of Cu in the body is in this form)
What normally happens to Ceruloplasmin in the body?
It goes to the blood, then comes back to the liver to be excreted in the bile.
What happens to Ceruloplasmin in Wilson’s disease?
It never leaves the liver which causes accumulation of copper in the organ
What 3 major organs are affected by Wilson’s disease?
Liver, Brain, and the eyes
1) Liver cirrhosis
2) Brain damage
3) Eye damage
What type of symptoms are present in Wilson’s disease?
Neurological/ psychiatric and liver damage “Parkinson like”
The copper that accumulates in the eyes forms ______
A green ring around the pupil which is known as Kayser-Fleischer (between cornea and conjunctiva)
What is the life expectancy for Wilson’s disease?
20 - 25 years, death due to liver failure
What can Wilson’s disease be treated by?
D-penicillamine (penicillin) to reduce rate of absorption by the body but a liver transplant may be necessary
What is used to treat systemic sclerosis?
D-Penicillamine, however this drug has also an immunodepressant secondary effect (used in autoimmune diseases)
What is Dystrophic calcification?
Deposition of calcium salts in necrotic tissue
What is an example of Dystrophic Calcification?
Ghon’s pox
What is Ghon’s pox?
An area of inflammation and granuloma (Casseous necrosis)
What are the 2 types of Calcification?
Dystrophic and Metastatic
Where would you see Ghon’s pox?
You would see this in the lungs in a patient with primary tuberculosis
What is the most common infectious disease, and how does it develop?
Primary Tuberculosis, and it develops due to attempt to kill the mycobacterium bacilli (TB bacteria)
What is seen on an X ray in a patient with TB?
The tissue undergoes calcification and can be visualized in a chest X-ray. TB – Casseous necrosis in lungs – calcification in lungs
What is seen on an X ray in a patient with TB?
The tissue undergoes calcification and can be visualized in a chest X-ray. TB – Casseous necrosis in lungs – calcification in lungs
What are the 3 diseases associated with Dystrophic Calcification?
1 Ghon’s pox
2 Systemic sclerosis aka scleroderma
3 Valvular disease
Systemic sclerosis aka scleroderma is associated with what gangrene?
Dry gangrene
What is systemic sclerosis?
An autoimmune disease with overactivity of fibroblasts which produce CT in the skin
What does “Overproduction” mean in systemic sclerosis aka scleroderma?
It means there is too much CT in the body that is replacing functional tissue, thus making the tissue atrophic
What do the joints look like in regard to Systemic sclerosis?
Look normal and healthy, but the function is decreased due to the shrinking of functional tissue (no ROM). Deposition of calcium is seen on X rays
What do the fingers undergo in Systemic sclerosis?
Sclerodactily (claw hands) and even gangrene (dry gangrene)
What does the face look like in patients with Systemic sclerosis?
Unable to express emotions
What does Valvular disease cause?
It causes atrophy of the valves and the deposition of calcium
Where is Valvular disease found?
Seen in Rheumatic fever and it is a form of (Fibrinoid necrosis)
What is the definition of Metastatic Calcification?
Deposition of calcium salt in organs or tissues, other than that which is atrophic or necrotic
What is Metastatic calcification associated with?
Hypercalcemia.
- non-necrotic, non-atrophic, and non-dystropic
What are the 6 causes of Metastatic Calcification?
1) Increased secretion of PTH
2) Destruction of bone
3) Vitamin D related disorders
4) Sarcoidosis
5) Renal failure in Secondary HPT
6) Kidney stones
What are the 3 categories under “Destruction of bone” in Metastatic Calcification?
1) Accelerated turnover (Paget’s)
2) Immobilization
3) Tumors (Multiple Myeloma, Leukemia, Multiple skeletal metastasis)
What are the 3 ways tissue is Vulnerable to Injury?
1) Ischemia
2) Ionizing Radiation
3) Viral Infection
What is Ischemia?
Lack of blood supply to an organ or tissue
What areas of the body are extremely vulnerable to ischemia and hypoxia?
Brain and Myocardium. Brain needs 20% of the oxygen
If ischemia occurs in the brain, what happens?
STROKE (liquefaction)
If ischemia occurs in the heart what happens?
Myocardial infarction (coagulation)
What organ is the least vulnerable to Ischemia?
Liver (its very resistant)
What other organs are also vulnerable to ischemia?
Lung, kidney and spleen (not as much as heart and brain though)
What is Hypoxia?
Lack of oxygen to tissue
Why is Ionizing Radiation vulnerable to tissue?
Labile cells (cells that are multiplying) aka HIGH MITOTIC RATE organs suffer the most
What are the organs that suffer the most from Ionizing Radiation?
- Reproductive cells (gonads)
- Bone marrow
- Hair Follicles
- Epithelia Cells and mucous membrane of GI tract ***
What does ionizing radiation lead to?
Destruction of DNA and therefore impairs the DNA replication
What cells are MOST VULNERABLE to Ionizing Radiation?
Epithelial cells of the GI tract. When Blood cells are affected it can lead to Leukemia
What is the LEAST vulnerable tissue in ionizing radiation?
Bone
