WM is an inert and slowly progressing disease, and its prognosis is very different. The median survival time is mostly 5 ~ 7 years. All the elderly men lost weight with coagulation disorder, decreased serum albumin, decreased blood cell count, and higher serum β2-MG and IgM. 40 g/L and obvious hyperviscosity are related to poor prognosis.
Regarding the treatment of WM, the 2nd WM International Symposium proposed that alkylating agents (such as chlorambucil), nucleoside analogues (cladribine or fludarabine) and monoclonal antibody rituximab should be the ideal first-line choice for patients with newly diagnosed WM. However, when choosing the first-line treatment plan, comprehensive evaluation and individualized selection should be made according to the patient's age, whether there is cytopenia and whether autologous bone marrow transplantation is needed. If autologous transplantation is considered, alkylating agents and nucleoside analogues should be used with caution.
Because CD20 antigen is widely expressed in WM tumor cells, the application of anti-CD20 monoclonal antibody (rituximab) combined with nucleoside analogues (cladribine or fludarabine) should be a satisfactory treatment scheme (we have treated 2 cases with satisfactory results).