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Oligosecretory Myeloma With Amyloidosis and Alopecia

Amyloidosis is a systemic illness characterized by the extracellular deposition of abnormal proteins in body tissues and organs. In addition to renal involvement, amyloidosis can also present with a variety of skin manifestations, though rarely with alopecia. Sixteen cases of alopecia secondary to s...

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Autores principales: Bilal, Anum, Der Mesropian, Paul, Lam, Franklin, Shaikh, Gulvahid
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5788141/
https://www.ncbi.nlm.nih.gov/pubmed/29399587
http://dx.doi.org/10.1177/2324709617752737
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author Bilal, Anum
Der Mesropian, Paul
Lam, Franklin
Shaikh, Gulvahid
author_facet Bilal, Anum
Der Mesropian, Paul
Lam, Franklin
Shaikh, Gulvahid
author_sort Bilal, Anum
collection PubMed
description Amyloidosis is a systemic illness characterized by the extracellular deposition of abnormal proteins in body tissues and organs. In addition to renal involvement, amyloidosis can also present with a variety of skin manifestations, though rarely with alopecia. Sixteen cases of alopecia secondary to systemic amyloidosis are reported. There is one reported case that presented with alopecia universalis. We report a case of a 68-year-old woman presenting with alopecia universalis, rapid decline in kidney function, and nephrotic syndrome who was found to have multiple myeloma-associated AL amyloidosis (immunoglobulin light chain). Her serological workup including serum electrophoresis was negative and she underwent renal biopsy. Pathology revealed eosinophilic material within the mesangium that was Congo-red positive, had apple-green birefringence under polarized light, and ultramicroscopically appeared as fibrillary material. Subsequent bone marrow examination showed a diffuse increase in plasma cells with atypia indicating plasma cell neoplasm. This case underlines several interesting aspects of multiple myeloma and the way it may present with amyloidosis. The lack of monoclonal spike on electrophoresis yet positive light chain analysis deserves special attention by clinicians to avoid a missed diagnosis. The extensive skin involvement also raises several questions regarding the pathologic mechanisms of alopecia in a patient with amyloidosis.
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spelling pubmed-57881412018-02-02 Oligosecretory Myeloma With Amyloidosis and Alopecia Bilal, Anum Der Mesropian, Paul Lam, Franklin Shaikh, Gulvahid J Investig Med High Impact Case Rep Case Report Amyloidosis is a systemic illness characterized by the extracellular deposition of abnormal proteins in body tissues and organs. In addition to renal involvement, amyloidosis can also present with a variety of skin manifestations, though rarely with alopecia. Sixteen cases of alopecia secondary to systemic amyloidosis are reported. There is one reported case that presented with alopecia universalis. We report a case of a 68-year-old woman presenting with alopecia universalis, rapid decline in kidney function, and nephrotic syndrome who was found to have multiple myeloma-associated AL amyloidosis (immunoglobulin light chain). Her serological workup including serum electrophoresis was negative and she underwent renal biopsy. Pathology revealed eosinophilic material within the mesangium that was Congo-red positive, had apple-green birefringence under polarized light, and ultramicroscopically appeared as fibrillary material. Subsequent bone marrow examination showed a diffuse increase in plasma cells with atypia indicating plasma cell neoplasm. This case underlines several interesting aspects of multiple myeloma and the way it may present with amyloidosis. The lack of monoclonal spike on electrophoresis yet positive light chain analysis deserves special attention by clinicians to avoid a missed diagnosis. The extensive skin involvement also raises several questions regarding the pathologic mechanisms of alopecia in a patient with amyloidosis. SAGE Publications 2018-01-24 /pmc/articles/PMC5788141/ /pubmed/29399587 http://dx.doi.org/10.1177/2324709617752737 Text en © 2018 American Federation for Medical Research http://creativecommons.org/licenses/by/4.0/ This article is distributed under the terms of the Creative Commons Attribution 4.0 License (http://www.creativecommons.org/licenses/by/4.0/) which permits any use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Case Report
Bilal, Anum
Der Mesropian, Paul
Lam, Franklin
Shaikh, Gulvahid
Oligosecretory Myeloma With Amyloidosis and Alopecia
title Oligosecretory Myeloma With Amyloidosis and Alopecia
title_full Oligosecretory Myeloma With Amyloidosis and Alopecia
title_fullStr Oligosecretory Myeloma With Amyloidosis and Alopecia
title_full_unstemmed Oligosecretory Myeloma With Amyloidosis and Alopecia
title_short Oligosecretory Myeloma With Amyloidosis and Alopecia
title_sort oligosecretory myeloma with amyloidosis and alopecia
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5788141/
https://www.ncbi.nlm.nih.gov/pubmed/29399587
http://dx.doi.org/10.1177/2324709617752737
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