Systemic light chain amyloidosis myopathy responsive to daratumumab monotherapy.
Eur J Neurol
; 30(3): 745-748, 2023 03.
Article
em En
| MEDLINE
| ID: mdl-36403110
ABSTRACT
BACKGROUND AND PURPOSE:
Amyloid myopathy is a rare and severe manifestation of systemic light chain (AL) amyloidosis. Early diagnosis and staging are mandatory for optimal therapy, given the rapid progression of muscle weakness. Despite the efficacy of bortezomib-based treatment regimens, there is a lack of therapeutic alternatives in non-responsive patients.METHOD:
The case report of a patient with systemic AL amyloidosis myopathy treated with daratumumab is presented.RESULTS:
A 70-year-old man displayed severe proximal muscle weakness which had developed over a 10-month period. Blood tests revealed an immunoglobulin A lambda monoclonal gammopathy, whilst muscle biopsy showed amyloid deposits within the arteriolar walls, confirming the diagnosis of amyloid myopathy associated with AL amyloidosis. Initial treatment with a bortezomib-based regimen showed no clinical or hematological improvement. After switching to daratumumab monotherapy, our patient achieved a favorable evolution with respect to functional muscle scoring and a complete hematological response.CONCLUSION:
To our knowledge, this is the first case report of an amyloid myopathy showing a remarkable clinical improvement in response to daratumumab monotherapy. It thereby highlights the potential of daratumumab as a monotherapeutical approach to the treatment of amyloid myopathy complicating AL amyloidosis.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Amiloidose de Cadeia Leve de Imunoglobulina
/
Amiloidose
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Doenças Musculares
Tipo de estudo:
Screening_studies
Limite:
Aged
/
Humans
/
Male
Idioma:
En
Revista:
Eur J Neurol
Assunto da revista:
NEUROLOGIA
Ano de publicação:
2023
Tipo de documento:
Article
País de afiliação:
França