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Provedor de dados:  BJMBR
País:  Brazil
Título:  Henoch-Schönlein purpura nephritis associated with monoclonal gammopathy of renal significance: a case report
Autores:  Zhao,Hui
Huang,Wen-hui
Huang,Jun-yue
Lu,Shou-yan
Yang,Ya-hong
Ma,Zhi-gang
Data:  2019-01-01
Ano:  2019
Palavras-chave:  Monoclonal gammopathy of renal significance
Henoch-Schönlein purpura nephritis
Chemotherapy
Resumo:  Monoclonal gammopathy of renal significance (MGRS) can present with different morphologic features and lead to kidney failure. The Henoch-Schönlein purpura nephritis (HSPN) that cannot be relieved by treatment with glucocorticoid and immunosuppressive agents suggests the presence of monoclonal gammopathy in adult patients. The present study reports on a single case of HSPN associated with IgA-κMGRS. The patient who suffered from recurrent skin purpura for 6 months and nephrotic syndrome for 2 months was admitted to our hospital. Bone marrow biopsy showed monoclonal gammopathy of undetermined significance. Kidney biopsy indicated a Henoch-Schönlein purpura nephritis (HSPN, ISKDC classified as type III) with positive staining with κ-light chain in the glomeruli and renal tubular epithelial cells. Furthermore, skin biopsy showed leukocytoclastic vasculitis and negative staining for Congo red and light chain. Given both the renal and cutaneous involvement, the patient was considered to have HSPN associated with IgA-κMGRS. The patient experienced an exacerbation in his purpura-like lesions and clinical status after treatment with glucocorticoid and immunosuppressive agents. Consequently, the patient was put on a regimen that included dexamethasone (20 mg on the 1st, 4th, 8th, and 11th days of each month, iv) and bortezomib (2.4 mg on the 1st, 4th, 8th, and 11th days of each month, iv). Eight weeks after treatment, he had complete resolution of his cutaneous purpura and his biochemical parameters improved. The latent presence of MGRS in cases of HSPN should be considered in adult patients. Increased cognizance and correct treatment options could improve patient outcomes.
Tipo:  Info:eu-repo/semantics/report
Idioma:  Inglês
Identificador:  http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2019000700501
Editor:  Associação Brasileira de Divulgação Científica
Relação:  10.1590/1414-431x20198222
Formato:  text/html
Fonte:  Brazilian Journal of Medical and Biological Research v.52 n.7 2019
Direitos:  info:eu-repo/semantics/openAccess
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