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Pulmonary AL amyloidosis: A review and update on treatment options.

ABSTRACT: Amyloidosis is a rare disease that involves the extracellular deposition of abnormally folded proteins, precipitating organ dysfunction. Pulmonary amyloidosis is frequently characterized by the AL amyloid subtype and can be localized or associated with systemic involvement, presenting in a nodular, diffuse alveolar-septal, or tracheobronchial pattern. Presentation of disease can vary from clinically silent to severe. Pulmonary amyloidosis is typically first suspected on CT scan of the chest. Diagnostic workup requires tissue biopsy and identification by immunohistochemical staining. Systemic treatment has evolved over recent years to include the combination of daratumumab, bortezomib, cyclophosphamide, and dexamethasone (dara-VCD) as first-line therapy, with the goal of quickly attaining complete hematologic response. Through clinical vignettes, we review pulmonary AL amyloidosis and discuss current treatment options.

SUBMITTER: Moy LN

PROVIDER: S-EPMC9287433 | biostudies-literature | 2022 Aug

REPOSITORIES: biostudies-literature

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Pulmonary AL amyloidosis: A review and update on treatment options.

Moy Lindsay N LN Mirza Mahum M Moskal Blake B Asado Nahren N Shah Bhaven B Bitran Jacob J

Annals of medicine and surgery (2012) 20220708

Amyloidosis is a rare disease that involves the extracellular deposition of abnormally folded proteins, precipitating organ dysfunction. Pulmonary amyloidosis is frequently characterized by the AL amyloid subtype and can be localized or associated with systemic involvement, presenting in a nodular, diffuse alveolar-septal, or tracheobronchial pattern. Presentation of disease can vary from clinically silent to severe. Pulmonary amyloidosis is typically first suspected on CT scan of the chest. Dia ...[more]

PMID: 35855884

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