Amyloses

The pathology in all forms of amyloidosis involves the extra cellular deposition of protein as characteristic fibrillar aggregates which interfere with tissue structure and function. Amyloid fibrils are derived from different unrelated proteins in the different forms of the disease. Amyloidosis may be hereditary or acquired, and the deposit distribution may be focal, localized or systemic. The diagnosis of amyloidosis requires histological confirmation usually with Congo red staining. Prognosis depends upon the degrees of visceral amyloid deposition, particularly in the heart. A diagnosis of amyloidosis should be followed by characterization of the fibril protein type in order to determine the appropriate management. The aim of treatment is to reduce the supply of the respective amyloid fibril precursor protein, which can result in major regression of deposits.