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Cutaneous amyloidosis as the first presentation of Waldenstrom macroglobulinemia

BACKGROUND: Waldenstrom macroglobulinemia is a lymphoplasmacytic lymphoma with elevated serum immunoglobulin M and multi-organ involvement. Primary systemic amyloidosis usually develops due to immunoglobulin light chains depositions in different organs due to an underlying gammopathy. CASE PRESENTAT...

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Detalles Bibliográficos
Autores principales: Rafiei, Rana, Eftekhari, Hojat, Rafiee, Behnam
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Babol University of Medical Sciences 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7442455/
https://www.ncbi.nlm.nih.gov/pubmed/32874445
http://dx.doi.org/10.22088/cjim.11.3.340
Descripción
Sumario:BACKGROUND: Waldenstrom macroglobulinemia is a lymphoplasmacytic lymphoma with elevated serum immunoglobulin M and multi-organ involvement. Primary systemic amyloidosis usually develops due to immunoglobulin light chains depositions in different organs due to an underlying gammopathy. CASE PRESENTATION: Our patient was an 86-year-old man with macroglossia, ecchymotic patches and bullous lesions associated with a skin laxity on the periorbital, palmar, and glans penis areas. Skin biopsy confirmed dermal amyloid depositions. In serum immunofixation electrophoresis, prominent monoclonal immunoglobulin-M lambda light chains were detected associated with prominent lymphoplasmacytic infiltration in bone marrow biopsy which was diagnosed as Waldenstrom macroglobulinemia. CONCLUSION: Skin involvement presenting as cutaneous amyloidosis could be the first manifestation of Waldenstrom macroglobulinemia. We should think about an underlying gammopathy in an old patient with skin laxity and ecchymosis.