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Journal: I.P. Pavlov Russian Medical Biological Herald (Vol.24, No. 3)

Publication Date:

Authors : ; ; ;

Page : 141-153

Keywords : systemic AL-amyloidosis; multiple myeloma; restrictive cardiomyopathy; chronic heart failure with preserved ejection fraction;

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As a result of somatic mutations, an abnormal clone of bone marrow plasma cells is formed in AL-amyloidosis. They produce amyloidogenic light chains of immunoglobulins, which are deposited in the form of insoluble amyloid fibrils in tissues of target organs. The clinical picture of AL-amyloidosis is diverse and determined by a primary involvement of various organs into the pathological process. One of the most severe manifestations is heart involvement. Diagnosis of amyloidosis is based on AL-detection of monoclonal paraprotein by means of immunoelectrophoresis of serum and urine proteins, and also quantifying free light chains of immunoglobulins in the serum. The results of biopsy with histological examination play the decisive role in the formulation of a definitive diagnosis. The goal of the treatment is to inhibit the proliferation of a plasma cells clone in order to reduce the production of immunoglobulin light chains. The clinical case of systemic amyloidosis associated with multiple myeloma is described.

Last modified: 2017-01-27 22:31:37