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Article
Pulmonary AL amyloidosis: A review and update on treatment options
Annals of medicine and surgery (2012)
  • Lindsay N Moy, Advocate Aurora Health
  • Mahum Mirza, Advocate Aurora Health
  • Blake Moskal, Advocate Aurora Health
  • Nahren Asado, Department of Pathology at ALGH, United States.
  • Bhaven Shah, Department of Pulmonology and Critical Care Medicine at ALGH, United States.
  • Jacob Bitran, Department of Hematology and Oncology at ALGH, United States.
Affiliations

Advocate Lutheran General Hospital

Scholarly Activity Date
8-1-2022
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.

Type
Article
PubMed ID
35855884
Citation Information

Moy LN, Mirza M, Moskal B, Asado N, Shah B, Bitran J. Pulmonary AL amyloidosis: A review and update on treatment options. Ann Med Surg (Lond). 2022;80:104060. Published 2022 Jul 8. doi:10.1016/j.amsu.2022.104060