Multiorgan involvement by amyloid light chain amyloidosis.
J Int Med Res
; 47(4): 1778-1786, 2019 Apr.
Article
em En
| MEDLINE
| ID: mdl-30803274
Amyloid light chain (AL) amyloidosis is a protein conformational disease. AL amyloidosis results from aggregation of misfolded proteins that are deposited in tissues as amyloid fibrils. Diagnosis of AL amyloidosis can be challenging due to its low incidence and clinical complexity. Therapy requires a risk-adapted approach involving dose reductions and schedule modifications of chemotherapy regimens along with close monitoring of hematologic and organ responses. We herein describe a patient whose condition was diagnosed as systemic AL amyloidosis and presented with splenic rupture as the initial symptom. Congo red staining of the kidney biopsy was positive. The normal structure of the liver and spleen had been replaced by amyloid deposition. The chemotherapy strategy involved a combination of bortezomib, cyclophosphamide, thalidomide, and dexamethasone.
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MEDLINE
Assunto principal:
Ruptura Esplênica
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Amiloidose de Cadeia Leve de Imunoglobulina
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Hepatopatias
Idioma:
En
Ano de publicação:
2019
Tipo de documento:
Article