Waldenström macroglobulinemia: 2023 update on diagnosis, risk stratification, and management - PubMed
Source : https://pubmed.ncbi.nlm.nih.gov/36588395/
Bortezomib, cyclophosphamide, fludarabine, thalidomide, everolimus, Bruton Tyrosine Kinase inhibitors, carfilzomib, lenalidomide, bendamustine, and venetoclax have all been shown to have activity in relapsed WM. Given WM's natural history, the reduction of therapy toxicity is an important part of tr ...
Conclusions: Management of refractory disease: Bortezomib, cyclophosphamide, fludarabine, thalidomide, everolimus, Bruton Tyrosine Kinase inhibitors, carfilzomib, lenalidomide, bendamustine, and venetoclax have all been shown to have activity in relapsed WM. Given WM's natural history, the reduction of therapy toxicity is an important part of treatment selection.
• Source: American Journal of Hematology
• Conclusion: “When WM is diagnosed before the development of symptoms, patients may be safely observed and monitored. However, patients with symptoms require chemotherapy.”
• Waldenström macroglobulinemia (WM) is a lymphoplasmacytic lymphoma characterized by the presence of immunoglobulin M (IgM) monoclonal protein. Clinical features of the disease include anemia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy, and rarely hyperviscosity.
• A diagnosis of WM is confirmed by the presence of IgM monoclonal protein associated with ≥10% clonal lymphoplasmacytic cells in bone marrow. More than 90% of patients harbor the L265P mutation in MYD88, and it is present in most IgM MGUS patients. Of note, MYD88 is not required for the diagnosis.
• Mayo Clinic–preferred treatment options for WM are rituximab and bendamustine or zanubrutinib, with a goal of minimizing toxicity secondary to therapy, as well as the avoidance of late complications.