Indication

Amyloid light-chain amyloidosis

Amyloid light-chain amyloidosis is a rare, systemic protein-misfolding disorder caused by an underlying clonal plasma cell dyscrasia. Read more about Amyloid light-chain amyloidosis

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About Amyloid light-chain amyloidosis.

Amyloid light-chain amyloidosis is a rare, systemic protein-misfolding disorder caused by an underlying clonal plasma cell dyscrasia.

The disease primarily affects older adults and is characterized by the overproduction of monoclonal immunoglobulin light chains that misfold into insoluble fibrillar deposits within the extracellular space of various organs.

Pathologic accumulation most frequently targets the heart and kidneys, although the peripheral nervous system, gastrointestinal tract, liver, and soft tissues are often involved.

Clinically, this process manifests as progressive multisystem organ dysfunction, prominently presenting with restrictive cardiomyopathy, nephrotic-range proteinuria, autonomic or peripheral neuropathy, and hepatomegaly.

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