Pulmonary AL amyloidosis: A review and update on treatment options.
AL amyloid
Pulmonary amyloidosis
Journal
Annals of medicine and surgery (2012)
ISSN: 2049-0801
Titre abrégé: Ann Med Surg (Lond)
Pays: England
ID NLM: 101616869
Informations de publication
Date de publication:
Aug 2022
Aug 2022
Historique:
received:
09
05
2022
revised:
21
06
2022
accepted:
22
06
2022
entrez:
20
7
2022
pubmed:
21
7
2022
medline:
21
7
2022
Statut:
epublish
Résumé
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.
Identifiants
pubmed: 35855884
doi: 10.1016/j.amsu.2022.104060
pii: S2049-0801(22)00820-2
pmc: PMC9287433
doi:
Types de publication
Journal Article
Review
Langues
eng
Pagination
104060Informations de copyright
© 2022 The Authors.
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