6
UNIT 1
Basic Principles of Cell Structure and Function
Alpha 1-Antitrypsin.
PASD (periodic acid-Schiff with diastase digestion),
3
173
Alpha 1-antitrypsin deF ciency is an autosomal recessive disorder characterized
by low serum concentrations of the enzyme
alpha 1-antitrypsin
, which inhibits
or inactivates proteases and elastases. Alpha 1-antitrypsin deF
ciency may cause
neonatal hepatitis with later cirrhosis and pulmonary panacinar emphysema.
Image:
A liver biopsy in a patient with alpha 1-antitrypsin deF ciency shows accu-
mulated alpha 1-antitrypsin as hyaline globules in this PAS stain. The globules
remain after the tissue has been treated with diastase (diastase resistant).
Amyloid.
H&E,
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173
Amyloid
is an abnormal protein caused by many pathological conditions, the most
common of which include
AL amyloid
, caused by light chains secreted by plasma
cells in plasma cell myeloma or monoclonal B-cell neoplasms;
AA amyloid
, seen
in chronic infl ammatory conditions; and
A
b
amyloid
in Alzheimer disease. Many
other types of amyloids exist.
Image:
A lymph node containing AL amyloid in a patient with small lymphocytic
lymphoma is shown. In H&E-stained preparations, amyloid is amorphous and
eosinophilic. Amyloid stained with Congo red shows a characteristic green bire-
fringence when viewed with polarized light.
Fatty Change.
H&E,
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173
Fatty change
, or
steatosis
, is the abnormal intracellular accumulation of lipid.
Although it can occur in many organs, it is most commonly seen in the liver because
of a variety of causes including alcohol abuse, hepatitis C, genetic predisposition,
medications, toxins, and diabetes mellitus.
Image:
This liver biopsy shows marked steatosis with intracellular lipid vacuoles.
Lewy Body.
Immunohistochemistry for alpha-synuclein,
3
431
A
Lewy body
is an intracytoplasmic oval with a halo, formed in neuromelanin-
containing neurons in patients with idiopathic Parkinson disease.
Image:
In this immunoperoxidase preparation, brown pigment indicates the pres-
ence of alpha synuclein (
arrow
), the main protein in the inclusion.
Neuro±
brillary Tangles.
Silver stain,
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173
In patients with Alzheimer disease, microtubule-associated protein tau and
abnormally
phosphorylated neuroF laments form
neurof brillary tangles
within neurons.
Image:
This silver-stained slide shows a twisted, black helix (
arrows
) in the cytoplasm
of a neuron from a patient with Alzheimer disease.
Cellular Accumulations
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