hematology and oncology
Macroglobulinemia
Key Points
Macroglobulinemia is a malignant plasma cell disorder in which B cells produce excessive amounts of IgM M-proteins. Most patients are initially asymptomatic, but many present with anemia or hyperviscosity syndrome (fatigue, weakness, skin and mucosal bleeding, visual disturbances, headache, peripheral neuropathy, and other neurologic manifestations). Do serum protein electrophoresis followed by serum and urine immunofixation, and quantitative immunoglobulin levels. Treat hyperviscosity using plasma exchange, which rapidly reverses bleeding as well as neurologic abnormalities. Corticosteroids,fludarabine,rituximab, proteasome inhibitors (bortezomibandcarfilzomib), immunomodulatory agents (thalidomide,lenalidomide, andpomalidomide), Bruton's tyrosine kinase inhibitors (ibrutinib,acalabrutinib, andzanubrutinib),idelalisib,everolimus, or the combination ofibrutinibandrituximabmay be helpful; other alkylating drugs can be used for palliation. 