TY - JOUR
T1 - Unique Renal Manifestation of Type I Cryoglobulinemia, With Massive Crystalloid Deposits in Glomerular Histiocytes, Podocytes, and Endothelial Cells
AU - Hoelbeek, Joris
AU - Duivenvoorden, Raphaël
AU - de Boer, Onno J.
AU - Claessen, Nike
AU - van den Bergh Weerman, Marius A.
AU - Roelofs, Joris J. T. H.
AU - Kers, Jesper
AU - ten Berge, Ineke J. M.
AU - Florquin, Sandrine
PY - 2016
Y1 - 2016
N2 - 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. In addition to the clinical examinations, light microscopy with immunohistochemistry, sequential multicolor immunohistochemistry, and electron microscopy were used to characterize the crystalline deposits. 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. This patient displays a unique renal manifestation of type I cryoglobulinemia related to a plasma cell dyscrasia
AB - 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. In addition to the clinical examinations, light microscopy with immunohistochemistry, sequential multicolor immunohistochemistry, and electron microscopy were used to characterize the crystalline deposits. 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. This patient displays a unique renal manifestation of type I cryoglobulinemia related to a plasma cell dyscrasia
U2 - https://doi.org/10.1093/ajcp/aqv027
DO - https://doi.org/10.1093/ajcp/aqv027
M3 - Article
C2 - 26796493
SN - 0002-9173
VL - 145
SP - 282
EP - 285
JO - American Journal of Clinical Pathology
JF - American Journal of Clinical Pathology
IS - 2
ER -