A CLINICAL CASE OF SYSTEMIC AL-AMYLOIDOSIS WITH PECULIAR DISEASE PRESENTATION
https://doi.org/10.24884/1561-6274-2017-21-6-78-85
Abstract
AL-amyloidosis is a disease associated with the clonal plasma cell proliferation and aberrant immunoglobulin light chain secretion. Deposition of insoluble polymers composed of monoclonal light chain lead to disruption of tissue architecture and organ dysfunction. Infiltrative amyloidogenesis often results in restrictive cardiomyopathy, leading to progressive heart failure, and kidney involvement with nephrotic proteinuria and dysfunction. Diagnostic is based on findings of tissue amyloid deposition and confirmation of monoclonal light chain nature. Here we reported course, diagnostic and successful treatment of primary AL-amyloidosis case with unusual disease presentation with lungs and pleura involvement, resulting further in heart and kidneys injuries.
About the Authors
S. A. Boldueva
North-Western State Medical University named after I.I. Mechnikov
Russian Federation
Russian Federation
Prof. SvetlanaA. Boldueva - MD, PhD, DMedSci., Prof., Department of Faculty Therapy.
195067, St-Petersburg, Piskarevsky ave. 47, (812) 543-15-71
D. V. Oblavatskii
North-Western State Medical University named after I.I. Mechnikov
Russian Federation
Russian Federation
Dmitry V. Oblavatsky – MD, Department of Cardiology for treatment of patients with myocardial infarction, cardiologist.
195067, St-Petersburg, Piskarevsky ave. 47, (812) 543-15-71
V. V. Grokhotova
North-Western State Medical University named after I.I. Mechnikov
Russian Federation
Russian Federation
VeraV. Grokhotova – MD, Department of Surgical Treatment of Cardiac Arrhythmias, arrhythmologist.
195067, St-Petersburg, Piskarevsky ave. 47, (812) 5431616
O. B. Bystrova
Nephrology Research Institute
Russian Federation
Russian Federation
Olga B. Bystrova – MD, nephrologist.
8(952)202-47-24
D. A. Mayer
Nephrology Research Institute
Russian Federation
Russian Federation
Dmitriy A. Mayer - clinical resident.
8(981)710-01-98
V. A. Dobronravov
Nephrology Research Institute
Russian Federation
Russian Federation
Vladimir A. Dobronravov - MD, PhD, DMedSci, Professor, research associate director of Nephrology Research Institute.
7(812)338-69-16
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Review
For citations:
Boldueva S.A., Oblavatskii D.V., Grokhotova V.V., Bystrova O.B., Mayer D.A., Dobronravov V.A. A CLINICAL CASE OF SYSTEMIC AL-AMYLOIDOSIS WITH PECULIAR DISEASE PRESENTATION. Nephrology (Saint-Petersburg). 2017;21(6):78-85. (In Russ.) https://doi.org/10.24884/1561-6274-2017-21-6-78-85
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