Journal
BLOOD CANCER JOURNAL
Volume 11, Issue 5, Pages -Publisher
SPRINGERNATURE
DOI: 10.1038/s41408-021-00483-7
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Funding
- NCI NIH HHS [P50 CA186781] Funding Source: Medline
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AL amyloidosis can present with symptoms such as nephrotic range proteinuria, heart failure, peripheral neuropathy, hepatomegaly, or diarrhea, and may develop from smoldering multiple myeloma and monoclonal gammopathy of undetermined significance (MGUS). Early diagnosis is crucial as these patients exhibit distinctive symptoms such as weight loss, edema, early satiety, and dyspnea.
Immunoglobulin light chain amyloidosis (AL) commonly presents with nephrotic range proteinuria, heart failure with preserved ejection fraction, nondiabetic peripheral neuropathy, unexplained hepatomegaly or diarrhea, and should be considered in patients presenting with these symptoms. More importantly, patients being monitored for smoldering multiple myeloma and a monoclonal gammopathy of undetermined significance (MGUS) are at risk for developing AL amyloidosis. MGUS and myeloma patients that have atypical features, including unexplained weight loss; lower extremity edema, early satiety, and dyspnea on exertion should be considered at risk for light chain amyloidosis. Overlooking the diagnosis of light chain amyloidosis leading to therapy delay is common, and it represents an error of diagnostic consideration. Herein we provide a review of established and investigational treatments for patients with AL amyloidosis and provide algorithms for workup and management of these patients.
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