Amyloidosis

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Related to AL amyloidosis: AA amyloidosis, Cardiac amyloidosis

amyloidosis

[‚am·ə·loi′dō·səs]
(medicine)
Deposition of amyloid in one or more organs of the body.

Amyloidosis

 

or amyloid dystrophy, a disorder of protein metabolism accompanied by the formation within tissues of a specific protein polysaccharide complex known as amyloid. The progress of the disease is associated with distortion of the protein-synthesizing function of the reticuloendothelial system and with accumulation of anomalous proteins in the blood plasma which act as autoantigens and also stimulate the formation of autoantibodies. As a result of antigen-antibody interaction, there is deposition of widely dispersed proteins which figure in the formation of amyloid. Once deposited in tissues, such as vascular and gland walls, the amyloid displaces functionally specialized elements of the organ; this process leads to the destruction of the organ.

Several types of amyloidosis are recognized: primary, secondary, and senile amyloidosis; amyloidosis with multiple myeloma; and localized tumorlike amyloidosis. Primary amyloidosis has no connection with any other disease; it affects mainly the cardiovascular system, alimentary tract, muscles, and skin. Secondary amyloidosis develops subsequent to other diseases which are accompanied by prolonged suppuration and tissue breakdown, such as tuberculosis, syphilis, and rheumatoid arthritis. It most often affects the spleen, liver, kidneys, adrenal glands, and intestines. Senile amyloidosis usually involves the heart. Amyloidosis may be either general or localized. Im-munodepressive and hepatic preparations are used in its treatment.

V. V. SEROV

References in periodicals archive ?
This is the first proteasome inhibitor and first investigational therapy for AL amyloidosis to receive Breakthrough Therapy designation.
He was referred to a haematology expert and bone marrow tests along with biopsies showed it was AL Amyloidosis.
Only around 30 people in the world have this strain, but he was eventually diagnosed with AL amyloidosis Dr Jonathan Wallace, a consultant haematologist at the Freeman hospital said: "AL amyloidosis is a serious condition that without treatment would be fatal".
4, 8) Gertz et al (10) in a series of 211 patients with AL amyloidosis reported that one-third of those with renal disease at presentation progressed to long-term dialysis dependence.
AL amyloidosis disability claims pending before or received after May 7, 2009, may be granted presumptive service-connection.
CMS said it analyzed the medical and scientific evidence and considered recommendations of professional societies and other experts in the field before making a determination that AuSCT can provide a health benefit for Medicare beneficiaries of any age with primary AL Amyloidosis.
The diagnosis of AL amyloidosis requires 1) demonstration of amyloid in tissue and 2) demonstration of a plasma cell dyscrasia.
As a result, this assay is now recommended for the initial evaluation of suspected myeloma; for prognosis of plasma cell dyscrasias; and for monitoring oligose-cretory myeloma and AL amyloidosis.
In 1856 Wilks published a paper on amyloidosis (lardaceous disease) and probably described the first patient with the primary type, now called AL amyloidosis (9, 10).
AL amyloidosis is a plasma cell disorder that affects bone marrow's ability to break down and recycle antibodies (proteins) through the body.
Therefore, all patients - including those with monoclonal gammopathy of unknown significance (MGUS) and asymptomatic MM (smouldering MM) patients - should have this test at the point of diagnosis; - Freelite allows for quantitative monitoring of patients: - in AL amyloidosis patients, serial serum FLC measurements are more accurate and sensitive than SPE and IFE alone;.