Primary Systemic Amyloidosis Associated with Smoldering Myeloma: A Case Report

Observation: Amyloidosis is a clinical disorder caused by extracellular and/or intracellular deposition of insoluble abnormal amyloid fibrils derived from the aggregation of misfolded plasma protein. Primary systemic amyloidosis (PSA) are typically developed from AL proteins that are λ light chains...

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Bibliographic Details
Published in:Journal of the Turkish Academy of Dermatology 2016-12, Vol.10 (4)
Main Authors: Kelekçi, Kıymet Handan, Güngör Yılmaz, Karakuzu, Ali, Karaca, Şemsettin, Ermete, Murat, Özdemirkıran, Füsun
Format: Article
Language:English
Subjects:
Multiple myeloma
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Summary:Observation: Amyloidosis is a clinical disorder caused by extracellular and/or intracellular deposition of insoluble abnormal amyloid fibrils derived from the aggregation of misfolded plasma protein. Primary systemic amyloidosis (PSA) are typically developed from AL proteins that are λ light chains of immunoglobulins. Amyloid fibrils in PSA are progressively collected in tissues and they disrupt the structure and function of organs.The most commonly affected organs are heart, kidneys, gastrointestinal system, liver, lung, peripheral and autonomic nervous system. Smoldering multiple myeloma (asymptomatic multiple myeloma) refers an increment of serum monoclonal protein level and plasma cells in the bone marrow, or both in the absence of as hypercalcemia, renal insufficiency, anemia, lytic bone lesions related to plasma-cell disorders. We presented herein, a case of PSA associated with smoldering myeloma because it’s rare disease and it’s interesting due to diagnosis is delayed before cutaneous finding are appeared.
ISSN:1307-394X
DOI:10.6003/jtad.16104c2