Abstract
Intracapillary monoclonal deposits disease is a rare kidney disorder defined by the presence of intracapillary monoclonal IgM deposits that completely obstruct glomerular capillary lumina. It is typically associated with Waldenstrom macroglobulinemia and significant levels of circulating monoclonal IgM. We describe a patient that presented with nephrotic range proteinuria, rapidly rising serum creatinine, and low levels of biclonal IgM. Kidney biopsy showed intracapillary monoclonal deposits disease with a membranoproliferative pattern and focal crescent formation. Subsequent bone marrow biopsy revealed marginal zone lymphoma. Despite treatment, the patient rapidly progressed to end-stage renal disease. Patients with indolent lymphomas and low levels of circulating clonal IgM may present with aggressive renal disease due to IgM deposition.
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