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: 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:
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Authors | Diana Maria Chitimus, Edouard Berling, Laurent Garderet, Nadia Venturelli, Edoardo Malfatti, François Jérôme Authier, Guillaume Nicolas, Pascal Laforêt, Claire Lefeuvre |
Journal | European journal of neurology
(Eur J Neurol)
Vol. 30
Issue 3
Pg. 745-748
(03 2023)
ISSN: 1468-1331 [Electronic] England |
PMID | 36403110
(Publication Type: Case Reports, Journal Article)
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Copyright | © 2022 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology. |
Chemical References |
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Topics |
- Male
- Humans
- Aged
- Immunoglobulin Light-chain Amyloidosis
(complications, drug therapy, diagnosis)
- Bortezomib
(therapeutic use)
- Amyloidosis
(complications, drug therapy, diagnosis)
- Muscular Diseases
(complications, drug therapy, diagnosis)
- Muscle Weakness
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