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 ?
Systemic AL amyloidosis (formerly primary amyloidosis) is a monoclonal plasma cell proliferative disorder in which monoclonal immunoglobulin light chains are deposited in tissues.
514 Now the ability of CMR detecting early cardiac involvement caused by AL amyloidosis is unknown.
He was referred to a haematology expert and bone marrow tests along with biopsies showed it was AL Amyloidosis.
The goal of treatment for AL amyloidosis in MM is to eradicate the plasma cell clone producing the amyloidogenic protein as rapidly as possible, thereby preventing ongoing amyloid deposition into tissues.
AL amyloidosis is only diagnosed in approximately six to 10 patients in the North East every year.
In this report, we describe a case of primary AL amyloidosis in a young patient with isolated kidney involvement and rapid progression to ESRD, treated successfully with melphalan and ASCT.
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.
Macroglossia with indented tongue is considered pathognomonic of AL amyloidosis.
Odjick wrote that two months earlier he was diagnosed with AL amyloidosis.