Blood and Lymphatic Cancer: Targets and Therapy (Aug 2022)
Differential Diagnosis of Waldenström’s Macroglobulinemia and Early Management: Perspectives from Clinical Practice
Abstract
Shashank Cingam,1 Surbhi Sidana2 1Division of Hematology and Oncology, University of New Mexico Comprehensive Cancer Center, Albuquerque, NM, 87102, USA; 2Division of BMT and Cell Therapy, Stanford University School of Medicine, Stanford, CA, 94305, USACorrespondence: Surbhi Sidana, Division of Blood and Marrow Transplant and Cellular Therapy, Stanford University School of Medicine, 300 Pasteur Drive, Room H0101c, Stanford, CA, 94305, USA, Tel +650 498-6000, Fax +650 725-3321, Email [email protected]: Waldenström’s Macroglobulinemia (WM) is a clonal B-lymphocyte neoplasm characterized by the presence of IgM monoclonal protein and ≥ 10% bone marrow involvement with lymphoplasmacytic cells. Several mature B-cell and plasma cell disorders can potentially produce monoclonal IgM immunoglobulin and hence, careful consideration of the differential diagnosis is vital. Clinico-pathological features, immunophenotype, and MYD88 mutation status help distinguish WM from other plasma cell and lymphoproliferative disorders. Treatment is only indicated in patients symptomatic from adenopathy or organomegaly, neuropathy, hyper viscosity, cryoglobulinemia, cold agglutinin disease, cytopenia’s or amyloidosis. Alkylators (cyclophosphamide, bendamustine) in combination with anti-CD20 antibodies and novel targeted agents including Bruton tyrosine kinase (BTK) inhibitors like ibrutinib are the mainstay of frontline treatment in symptomatic WM.Keywords: Waldenström’s Macroglobulinemia, lymphoplasmacytic lymphoma, WM, LPL
