Use of bortezomib in heavy-chain deposition disease: a report of 3 cases.
Am J Kidney Dis
; 64(1): 123-7, 2014 Jul.
Article
em En
| MEDLINE
| ID: mdl-24613055
ABSTRACT
Heavy-chain deposition disease (HCDD) is a rare complication of plasma cell dyscrasia in which monoclonal heavy chains deposit in glomerular and tubular basement membranes of the kidney. Clinical and pathologic features of HCDD have been well described in case reports and series, but evidence supporting specific therapies is sparse. Historically, the disease has had a poor prognosis, intensifying the need to clarify optimal treatments. We describe 3 cases of HCDD with biopsy-proven glomerular involvement, severe nephrotic syndrome, and decline in kidney function that were treated successfully with bortezomib, a proteasome inhibitor. None of these patients had multiple myeloma. In all cases, bortezomib-based therapy resulted in sustained resolution of nephrotic syndrome and improvement in kidney function. All 3 patients developed peripheral neuropathy; otherwise, treatment was well tolerated. To our knowledge, this is the first description of the clinical effectiveness of bortezomib against HCDD.
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Base de dados:
MEDLINE
Assunto principal:
Pirazinas
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Ácidos Borônicos
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Doença das Cadeias Pesadas
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Antineoplásicos
Tipo de estudo:
Prognostic_studies
Limite:
Aged
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Humans
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Male
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Middle aged
Idioma:
En
Ano de publicação:
2014
Tipo de documento:
Article