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 ?
CAEL-101 is a first-in-class monoclonal antibody (mAb) designed to improve organ function by reducing or eliminating amyloid deposits in the tissues and organs of patients with AL amyloidosis. The antibody is designed to bind to insoluble light chain amyloid protein, including both kappa and lambda subtypes.
Uncommon presentations of AL amyloidosis can include alopecia and nail dystrophy (5-7).
Besides the presence of late gadolinium enhancement, high levels of N-terminal natriuretic peptide B, troponins, and free light chains, as well as the presence of pleural effusion, have all been found to be negative prognostic markers in AL amyloidosis [2, 5, 12].
Systemic AL amyloidosis (formerly primary amyloidosis) is a monoclonal plasma cell proliferative disorder in which monoclonal immunoglobulin light chains are deposited in tissues.
Monoclonal gammopathies can lead to the rare complication of AL amyloidosis in up to 2.2% of the patients [4].
Comparatively to AL amyloidosis ATTR has a more favorable prognosis of 3-5 years as it has a slower clinical course.
The widely global exchanged studies were those reports by Kyle and coworkers [24]; they found that the incidence for AL amyloidosis in United States was 9 per million person-years.
Assessment of cardiac function is important in the diagnostic work-up therapeutic follow-up and prognosis of AL amyloidosis as well as in the clinical management of these patients.
He was referred to a haematology expert and bone marrow tests along with biopsies showed it was AL Amyloidosis.
Two global phase 3 trials, TOURMALINE-MM1, to investigate MLN9708 in combination with lenalidomide and dexamethasone in relapsed and/or refractory multiple myeloma and TOURMALINE-AL1, to investigate MLN9708 plus dexamethasone in patients with relapsed or refractory light chain AL amyloidosis were initiated in 2012.
In a retrospective study of 19 patients with AL amyloidosis and 20 with AA amyloidosis, which required dialysis and were followed up after approximately 35 months, 15 (79 % ) with AL and 3 patients (15 %) with AA amyloidosis died10.
Outcome in systemic AL amyloidosis in relation to changes in concentration of circulating free immunoglobulin light chains following chemotherapy.