Successful treatment of IgM-monoclonal gammopathy of undetermined significance associated with cryoglobulinemia and cold agglutinin disease with immunochemotherapy with rituximab, fludarabine, and cyclophosphamide.

摘要

A 69-year-old woman with peripheral coldness as well as pain, numbness of the hands and feet, and purpura on the extremities on exposure to cold stimulation was referred to us. Physical examination found livedo reticularis on the distal extremities (Fig. la), Raynaud's phenomenon, and acrocyanosis. Laboratory investigations revealed elevated levels of IgM (698 mg dl"1), a markedly increased titer of cold agglutinin (>x 10,240), positive for the cyoglobulin test and direct Coombs test (2+(c3d), l+(IgG)) in serum, immeasurable red blood cell (RBC) count with rouleau formation, and negative for hepatitis C virus antibody and rheumatoid factor. We detected monoclonal IgM-K protein by immunoelectrophoretic study. Bone marrow study revealed that normal cellularity with a few atypical lymphocytes infiltrated in the morphology under light microscopy.