Pulmonary AL amyloidosis: A review and update on treatment options

Authors

Lindsay N. Moy, Advocate Aurora HealthFollow
Mahum Mirza, Advocate Aurora HealthFollow
Blake Moskal, Advocate Aurora HealthFollow
Nahren Asado, Department of Pathology at ALGH, United States.Follow
Bhaven Shah, Department of Pulmonology and Critical Care Medicine at ALGH, United States.Follow
Jacob Bitran, Department of Hematology and Oncology at ALGH, United States.Follow

Recommended Citation

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

Affiliations

Advocate Lutheran General Hospital

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