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Cryoglobulins (CGs) are immunoglobulins that precipitate at temperatures below 37 °C and dissolve again after rewarming. Cryoglobulinemia may be asymptomatic or cause end-organ damage by CG precipitation in small- to medium-sized blood vessels.1 In their seminal work, Brouet et al.2 classify cryoglobulinemias into 3 subgroups according to CG composition and clonality. In type II cryoglobulinemia there is a mixture of monoclonal IgM with rheumatoid factor activity and polyclonal IgG. In type III, CGs consist of polyclonal IgM and IgG.1 Type II and III cryoglobulinemias are also referred to as mixed cryoglobulinemias and are often caused by chronic hepatitis C infection and less frequently by autoimmune diseases or other viral infections (hepatitis B infection, HIV).3  CGs in type I cryoglobulinemia are monoclonal Igs (MIg), also known as paraproteins, commonly IgG, IgM subtypes, or free light chains. The underlying pathological process is a plasma cell or B-cell lymphoproliferative disease, such as multiple myeloma (MM), Waldenstrom macroglobulinemia, chronic lymphocytic leukemia, or other B-cell non-Hodgkin lymphoma. However, in approximately 40% of symptomatic cases, the plasma cell or B-cell clone is too small to fulfill the diagnostic criteria of MM or overt lymphoma. The term monoclonal gammopathy of undetermined significance (MGUS) used for these cases is a misnomer, as the MIg causes disease regardless of the size and tumor burden.4 For cases with renal involvement, the International Kidney and Monoclonal Gammopathy Research Group introduced the term monoclonal gammopathies of renal significance (MGRS).5 The updated MGRS definition includes monoclonal gammopathies that cause renal disease but have low tumor burden and thus treatment from the hematological standpoint is not imminently indicated.6 These patients may have fewer than 10% plasma cells in bone marrow biopsy, smoldering myeloma, or low-grade lymphomas.7 MGRSs are not of undetermined significance, and their relevance to renal pathology has opened the use of clone-directed therapies targeting the nephrotoxic MIg-producing clone, with an aim to preserve renal function.8 Retrospective studies to date suggest that clone-directed therapy improves renal outcomes.9  MGRS encompasses a wide spectrum of renal histopathological entities caused by a nephrotoxic MIg, including ever rarer subtypes such as type I cryoglobulinemic glomerulonephritis.S1,S2 Historically, MGRS may have gone underdiagnosed and patients not offered optimal treatment, unlike their counterparts fulfilling consensus myeloma or lymphoma diagnostic criteria.S3 Here, we report a case of severe type I crystal cryoglobulinemic glomerulonephritis caused by MGRS, for which treatment with myeloma induction therapy and autologous stem cell transplantation (ASCT) induced long-lasting complete hematological response and renal function preservation.

kidney international reports

Autologous Stem Cell Transplant for the Treatment of Type I Crystal Cryoglobulinemic Glomerulonephritis Caused by Monoclonal Gammopathy of Renal Significance (MGRS)