Abstract

Amyloidosis is a rare systemic disease of protein misfolding. The most common type of amyloidosis is light chain (AL) amyloidosis, in which the amyloidogenic precursor protein is a monoclonal immunoglobulin (Ig) light chain. Very few patients with AL amyloidosis have underlying IgM paraproteinemia. Neuropathy is a common manifestation of AL amyloidosis but is rarely seen as presenting manifestation. Association of amyloidosis with brachial plexopathy has been reported very rarely. Due to its rarity, IgM AL amyloidosis remains poorly studied. Herein, we report a very rare case of IgM related amyloidosis (AL) presenting as brachial plexopathy in 63-year-old male presented with chief complaint of severe fatigue, decreased appetite and significant weight loss and progressive right side upper limb weakness for last one year. Special staining with Congo Red Stain and immunohistochemical staining were further carried out and the diagnosis of systemic amyloidosis was made. Thorough Laboratory tests including protein electrophoresis, molecular test like CXCR-4 Mutation Analysis, MYD 88 (L265P) Mutation assay, Multiple Myeloma FISH Panel were performed to confirm the above diagnosis.

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