The World Health Organization defines Waldenstr?m macroglobulinemia (WM) as a lymphoplasmacytic lymphoma associated with a mono-clonal immunoglobulin M (IgM) protein. 1
Although believed to be a sporadic disease, studies have shown increased susceptibility within families, indicating a genetic component. Mutations in gene MYD88 and CXCR4 have been found to occur frequently in patients. WM cells show only minimal changes in cytogenetic and gene expression studies. Their miRNA signature however differs from their normal counterpart. It is therefore believed that epigenetic modifications play a crucial role in the disease. 1
Waldenstrom macroglobulinemia is slow growing and may not cause signs and symptoms for many years. Symptoms can be related to cytopenias, tumoural involvement, or IgM-related disorders. Clinical features include weakness, fatigue, anemia, weight loss, bleeding, fever, numbness in your hands or feet, changes in vision，thrombocytopenia, hepatosplenomegaly, lymphadenopathy, and rarely hyperviscosity,etc. 1,2
Not all patients who fulfill WM criteria require therapy; these patients can be observed until symptoms develop. The panel concluded that therapeutic strategies in WM should be based on individual patient and disease characteristics. When hyperviscosity is present, plasma exchange should be considered a temporizing measure until systemic therapy successfully lowers the tumor mass and thereby reduces the IgM protein concentration in the serum. The treatment recommendations including the BTK inhibitors, CD20 monoclonal antibody-based regimens, proteinase inhibitor -based regimens, alkylating agent and stem cell transplantation, etc. 1,2
1. Hematology Oncology Committee of China Anti-Cancer Association; Leukemia & Lymphoma Group Society of Hematology at Chinese Medical Association; Union for China Lymphoma Investigators. The consensus of the diagnosis and treatment of lymphoplasmacytic lymphoma/Walderstr?m macroglobulinemia in China (2016 version). Zhong hua Xue Ye Xue Za Zhi,2016,37(9):729-734.
2. NCCN. The NCCN guidelines for Waldenstr?m's Macroglobulinemia / Lymphoplasmacytic Lymphoma (version 2.2019) [EB/OL]. Fort Washington: NCCN,2019.https://www.nccn.org/professionals/physician_gls/pdf/waldenstroms.pdf.
CP-87244 Approved date 2019-4-28