Treatment of Cryoglobulinemia Type 1 with IgG Monoclonal Gammopathy of Undetermined Significance (MGUS)

Type I cryoglobulinemia is driven by a single clonal immunoglobulin that precipitates in the cold. When the underlying cause is IgG monoclonal gammopathy of undetermined significance (MGUS), the treatment target is the plasma-cell clone producing that immunoglobulin.

Clinical scenario

This protocol addresses patients with Type I cryoglobulinemia in the setting of IgG monoclonal gammopathy of undetermined significance (MGUS). IgG MGUS reflects clonal plasma-cell proliferation as the source of the pathogenic cryoglobulin, which distinguishes the therapeutic approach from cases driven by lymphoplasmacytic disease.

Treatment approach — partial overview

Treatment is directed at the underlying plasma-cell clone using myeloma-directed therapy. In addition, avoidance of cold-temperature exposure is a core component of management, as cold exacerbates cryoglobulin precipitation. The complete agent selection, sequencing, and clinical decision algorithm are available in the full protocol.

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References

DOI: 10.1016/j.jbspin.2019.01.016

IgG MGUS, which indicates plasma cell proliferation, is treated with those myeloma drugs that target the plasma cells, whereas rituximab is generally preferred for IgM MGUS, which indicates lymphoplasmacytic proliferation.
Thus, lymphoma requires combination chemotherapy and myeloma treatment with drugs such as bortezomib, thalidomide, lenalidomide, or alkylating agents.
Exposure to low temperatures exacerbates cryoglobulin formation and should therefore be avoided.

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