Joris Hoelbeek 1 , Raphaël Duivenvoorden 2 , Onno J de Boer 3 , Nike Claessen 3 , Marius A Van den Bergh Weerman 3 , Joris J T H Roelofs 3 , Jesper Kers 3 , Ineke J M Ten Berge 2 , Sandrine Florquin 3 Show Affiliations »
Abstract
OBJECTIVES: We describe a 62-year-old woman with a 15-year history of a plasma cell dyscrasia (monoclonal IgGκ), manifested by type I cryoglobulinemia and dermal vasculitis. METHODS: In addition to the clinical examinations, light microscopy with immunohistochemistry, sequential multicolor immunohistochemistry, and electron microscopy were used to characterize the crystalline deposits. RESULTS: At initial presentation and for a later flare, she was treated with cyclophosphamide and prednisolone with good clinical response. She had renal function decline, microscopic hematuria, and proteinuria. A renal biopsy specimen revealed the presence of glomerular macrophages and duplication of the capillary walls with cellular interposition. Glomerular cells contained abundant needle-shaped eosinophilic crystalline inclusions positive for κ light chain. Electron microscopy confirmed the presence of intracytoplasmatic crystalline structures in endothelial cells, podocytes, and macrophages but not in the tubular epithelium. Rituximab treatment was started. At follow-up (now up to 6 months), renal function remained stable. CONCLUSIONS: This patient displays a unique renal manifestation of type I cryoglobulinemia related to a plasma cell dyscrasia. © American Society for Clinical Pathology, 2016. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.
OBJECTIVES: We describe a 62-year-old woman with a 15-year history of a plasma cell dyscrasia (monoclonal IgGκ), manifested by type I cryoglobulinemia and dermal vasculitis . METHODS: In addition to the clinical examinations, light microscopy with immunohistochemistry, sequential multicolor immunohistochemistry, and electron microscopy were used to characterize the crystalline deposits. RESULTS: At initial presentation and for a later flare, she was treated with cyclophosphamide and prednisolone with good clinical response. She had renal function decline, microscopic hematuria , and proteinuria . A renal biopsy specimen revealed the presence of glomerular macrophages and duplication of the capillary walls with cellular interposition. Glomerular cells contained abundant needle-shaped eosinophilic crystalline inclusions positive for κ light chain. Electron microscopy confirmed the presence of intracytoplasmatic crystalline structures in endothelial cells, podocytes, and macrophages but not in the tubular epithelium. Rituximab treatment was started. At follow-up (now up to 6 months), renal function remained stable. CONCLUSIONS: This patient displays a unique renal manifestation of type I cryoglobulinemia related to a plasma cell dyscrasia . © American Society for Clinical Pathology, 2016. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.
Entities: Chemical
Disease
Species
Keywords:
Cryoglobulinemia; Crystalloid nephropathy; Crystals; Glomerulopathy
Mesh： See more »
Year: 2016
PMID： 26796493 DOI： 10.1093/ajcp/aqv027
Source DB: PubMed Journal: Am J Clin Pathol ISSN： 0002-9173 Impact factor: 2.493