Immunoglobulin M ‘Flare’ Seen in a Case of Waldenstrom’s Macroglobulinemia: Successfully Managed by Therapeutic Plasma Exchange

Therapeutic plasma exchange (TPE) is a conjunctive modality of treatment along with rituximab to decrease paraproteinemia associated with hyperviscosity. Here we narrate our experience in treating a diagnosed case of Waldenstrom’s macroglobulinemia in 70 years old male patient with moderate anemia and severe features of hyperviscosity syndrome by serial TPE and rituximab combined with bortezomib. The patient was relieved of his symptoms after initial two TPE procedures performed on alternative day. However he again developed signs and symptoms of the disease within 6 weeks following second TPE and starting of rituximab (375 mg/m² weekly for 4 weeks) therapy with bortezomib. His serum IgM level became as high as 9.901 g/dl suggesting immunoglobulin M ‘Flare’ due to rituximab therapy. At the end of third TPE he was relieved symptomatically with low IgM level (3.13 g/dl) and discharged in hemodynamically stable condition. Therefore we concluded that careful monitoring of serum viscosity and IgM level are necessary during treatment with rituximab based chemotherapy and TPE should be promptly initiated to control the treatment related hyperviscosity syndrome.