Amyloidosis

nature of amyloid
amyloid is a protienaceous substance deposited between
cells in various tissues and organs in a variety of clinical
settings
Starch like staining properties(PAS +ve)
Amorphous eosinophilic ,hyaline extracellular substance
seen under the microscope.
Although chemically diverse they share a physical property-
nonbranching long fibrils(7.5-10mm).by electron
diffraction,they are arranged –into beta-pleated sheaths.
Congo-red staining is typical, under polarized light they
show apple green birefringence.
PAS +ve substance
Congo red stain-apple green birefringence
chemical structure of amyloid
AL-Amyloid formed by -immunoglobulin light
chains.this form of amyloid is found in multiple
myeloma
AA- Amyloid associated protien synthesized by the
liver [ in the form of precursor serum amyloid A(SAA)]
A beta- Amyloid found in Alzheimer disease(Beta-
amyloid protien)
Amyloidosis-Defn
Amyloidosis is a group of diseases characterised by a
deposition of amyloid in various organs.
May occur as primary… or secondary form during
the course of a chronic inflammatory disease.
The disease maybe limited to a single organ ,or
systemic.
Some forms maybe hereditary
Primary amyloidosis
Systemic deposition of AL type amyloid

Assoc with plasma cell neoplasia and B cell

lymphoma

Monoclonal gammopathy(whole immunoglobulins

or only light chains)found by electrophoresis of serum
secondary amyloidosis
There is an underlying disease (eg
tuberculosis,chronic osteomyelitis,rheumatoid
arthritis and cancer) or condition(heroin abuse)

Widespread deposition of AA Protien

Amyloidosis of aging
Systemic deposition of amyloid in elderly patients
(seventh and eighth decade)
Type of amyloid –A-beta

Frequent involvement of heart(cardiac

amyloidosis)
systemic amyloidosis
Kidneys: typically enlarged ,pale and waxy.glomerular
mesangial, interstitial and vascular wall depositions of amyloid.
spleen: enlargement (splenomegaly ) with either nodular
depositions(sago spleen) or maplike(red pulp) depositions
(lardaceous spleen)
Liver: hepatomegaly,extracellular amyloid with pressure
atrophy of hepatocytes
Small blood vessels in many organs contain deposits of
amyloid.
Amyloid deposits in blood vessels can be observed in
gingival or rectal biopsies
sago spleen
lardaceous spleen
liver