Open Access Journal of Urology & Nephrology (OAJUN)

ISSN: 2578-4676

Case Report

Systemic Amyloidosis and the Challenge of the Treatment: Clinical Case

Authors: Bogov B*, Filev R, Lyubomirova M, Iliev A and Yanev K

DOI: 10.23880/oajun-16000183

Abstract

A 53-year-old female patient with primary amyloidosis and restrictive cardiomyopathy. For six years the patient has muscle weakness, joint and bone pain, periorbital subcutaneous hemorrhages, bullae on the lower eyelids, macroglossia, gradual thickening of the skin in the shoulder area, body and thighs. Also, there are subcutaneous hemorrhages in other parts of the body. The patient underwent immunofixation of urine and serum and the result showed presence of λ-light chain type and also of IgA. A few months before admission to the clinic, a right ureterolithotomy was performed. After evaluation of the patient, a specific treatment was conducted with melphalan and dehydrocortisone, with a scheme different from the combination regimens used worldwide. The different approach led to good response to the treatment, preserved the renal function and all the cytological tests of the bone marrow did not show plasma cell infiltration. Also, during the treatment, she had no significant proteinuria, no hypoproteinemia, but had persistent pleural effusions, pericardial effusion and persistent edema of the lower extremities.

Keywords: Primary Amyloidosis; Restrictive Cardiomyopathy; Melphalan; Dehydrocortisone; Chemotherapy; Immunomodulatory Agents

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