Cryoglobulinemia is the presence of circulating cryoglobulin which can cause systemic vasculitis and glomerulonephritis. Monoclonal gammopathy of renal significance is strongly associated with type I cryoglobulinemia, but the role of detectable serum monoclonal gammopathy in mixed (type II) cryoglobulinemia is not clearly established. We report a case of a 71-year-old woman who presented with skin rash, leg edema, and azotemia. Investigations showed a positive result for rheumatoid factor, low complement C4 level, positive result for serum cryoglobulin, and positive M-spike on serum protein electrophoresis and IgM kappa monoclonal gammopathy on serum immunofixation. Kidney biopsy revealed membranoproliferative glomerulonephritis, polytypic IgM-dominant deposits in an immunofluorescence study, and microtubular substructures in an electron microscopic study. After an extensive workup, no evidence of myeloma or lymphoma was found. A diagnosis of monoclonal gammopathy of renal significance–associated mixed cryoglobulinemic glomerulonephritis was made. Due to the detectable IgM kappa monoclonal gammopathy in the patient’s serum, clonal-directed therapy was administered. The patient had been in clinical remission after treatment with clone-directed therapy with cyclophosphamide and steroids. The literature review for cases of type II cryoglobulinemic glomerulonephritis that have detectable serum monoclonal gammopathy are summarized in this study.

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