JOURNAL ARTICLE

Unique Renal Manifestation of Type I Cryoglobulinemia, With Massive Crystalloid Deposits in Glomerular Histiocytes, Podocytes, and Endothelial Cells

Joris Hoelbeek, Raphaël Duivenvoorden, Onno J de Boer, Nike Claessen, Marius A Van den Bergh Weerman, Joris J T H Roelofs, Jesper Kers, Ineke J M Ten Berge, Sandrine Florquin
American Journal of Clinical Pathology 2016, 145 (2): 282-5
26796493

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.

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