4amyloidosis-Texts
4amyloidosis-Texts
4amyloidosis-Texts
Calcification &amyloidosis
The various agents which can cause cell
necrosis may also cause lesser cell damage
which is reversible when the injurious agent is
removed.
The appearances and effects are considered
under these headings:
1. hydropic swelling,
2. fatty change,
3. radiation
4. atrophy.
HYDROPIC SWELLING
Under the light microscope: Electron
microscopic (EM) appearances indicate the
mechanisms:
A) Damaged plasma membrane:
B) diminished ATP
C) diminished Na+ pump
D)Vacuolation of endoplasmic reticulum
E) Cell swells
Naked eye: The damaged organs become swollen
and are pale
FATTY CHANGE
This is accumulation of fat in non-fatty tissues, e.g. skeletal muscles and
the heart.
These tissues cannot metabolise the amount of lipid presented to them,
resulting in its accumulation within the cells. Examples include:
1- REDUCED cellular ENZYME activity
2- Inability to metabolise fat
3- Accumulationof fat in cells
EFFECTS OF FATTY CHANGE
Impairment of cellular function is usually due to the pathological process
causing the fatty change (e.g. anoxia in severe anaemia) and not to the
physical presence of fat within the cell
A possible exception is the myocardium
Fatty liver. A, The possible mechanisms leading to accumulation of
triglycerides in fatty liver.
B, High-power detail of fatty change of the liver.
xanthomas
CALCIFICATION
Abnormal deposits of calcium occur in 2
circumstances: dystrophic calcification and
metastatic
1. DYSTROPHIC CALCIFICATION
Local deposits of calcium may occur in:
(a) Necrotic tissue - old caseous lesions of
tuberculosis; old infarcts; collections of pus; in
fat necrosis associated with pancreatitis.
(b) Tissues undergoing slow degeneration -
hyaline areas in benign tumours in arteries due to
atheromatous degeneration or in old age; in old
thrombi; diseased or abnormal heart valves
METASTATIC CALCIFICATION
In this case there is an increase in the calcium
phosphate product in the blood (usually
hypercalcaemia)
Calcification of the aortic valve. A view looking down onto the
unopened aortic valve in a heart with calcific aortic stenosis.
fibroadenoma with calcification.
Amyloidosis
Fibrinoid, Hyalin
DEF.:
disorder of protein
metabolism accompanied
with abnormal
extracellular deposition of
proteinaceous material -
amyloid
Amyloid - history
Macroscopy:
Hereditary
AA - Familial Mediterranean Fever
ATTR - Famil. polyneuropatia
transthyretin (mutated form)
diminished functions of some organs, esp.
KIDNEY FAILURE
Senile cerebral
A - -amyloid
protein
Endocrine
ACal - ca medullare gl. thyreoideae
AIAPP - islets of Langerhans associated
AANF - isolated atrial amyloidosis
atrial natriuretic polypeptide
dysfunction)
Restrictive cardiomyopathy (predominant
diastolic
dysfunction)
Congestive heart failure
Rhytm abnormalities
Coronary insufficiency
Valvular dysfunction
Pericardial tamponade
Enhance sensitivity to digitalis glycosides
Atrial thrombosis - embolisation
Scintigraphy (in vivo)
using human serum amyloid component
marked with 123J
Echocardiography
(atrial amyloid)
Biochemistry
sequening DNA -hered. forms
extraction of fibrils (from a biopsy
specimen)
spectrometry
sequening of the amyloid protein
Macroscopy:
IRON-CONTAINING PIGMENT
Two main pigments are derived from the breakdown of red blood cells:
1. HAEMOSIDERIN
2. BILIRUBIN.
HAEMOSIDERIN
The iron derived from red cell breakdown is held in the spleen, liver
and bone marrow,
combined with apoferritin. In the plasma it is transported by
transferrin.
the amount of iron within the cells becomes excessive and overloads
the ferritin system, it is
deposited in a brown granular form
Hemosiderin granules in liver cells.
A, H&E section showing golden-brown, finely granular pigment.
B, Prussian blue reaction, specific for iron
Haemoglobin
Haematin
Lipofuscin
This is a yellowish brown pigment with a high
lipid content, often found in the atrophied
cells of old age – ‘wear and tear’ pigment. It is
particularly common in the heart muscle, and
the term ‘brown atrophy’ is often applied
Lipofuscin granules in a cardiac myocyte.
A, Light microscopy (deposits indicated by arrows).
B, Electron microscopy. Note the perinuclear, intralysosomal location.
EXOGENOUS PIGMENTATION