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Amyloidosis
Amyloidosis is an extracellular deposit mainly in the walls of blood
vessels and basement membranes and along reticulin fibers. It resembles
hyaline by light microscopy (homogenous esenophilic and refractive on
staining with haematoxylin and eosine) but can be differentiated from
it by special stains (Congo red).
Structure of amyloid: is formed of 90% fibril protein (many different
types) and 10% glycoprotein called P-component. Despite the different
types of amyloid present they all appear the same on biopsy.
Types of amyloidosis
Generalized (systemic) amyloidosis
- 1ry amyloidosis (B-cell dyscrasia): AL type of amyloid protein
resulting from fibril formation by monoclonal antibody light chains e.g.
multiple myeloma.
- 2ry amyloidosis (reactive systemic amyloidosis): systemic amyloidosis
of the AA type.
- Haemodyalisis associated amyloidosis: 70% of patients undergoing
haemodialysis due to deposition of B2 microglobulin which is not normally
filtered by the membrane.
- Hereditary amyloidosis: several types which may be localized or
generalized.
Localized amyloidosis
- Senile amyloidosis of the heart and choroid plexus.
- Within endocrine tumors.
- Idiopathic localized amyloid deposits.
Clinical manifestations
It can present with many varying manifestations according to the site
of involvement. However, when it affects the kidney it leads to nephrotic
syndrome.
Treatment
- No curative treatment for amyloidosis is available.
- Peripheral stem cell transplantation, melphalan and prednisone
are all under study and may be beneficial in some cases.
Conservative treatment for nephrotic syndrome
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