There are many different treatment options available for WM patients, and they are increasing as researchers discover more about the biology and genetics of the disease.
Treatment may consist of just one drug (single-agent therapy) or of two or more drugs (combination therapy). Most studies seem to indicate that combination therapies are more effective, resulting in better and/or longer-lasting responses.
A full discussion of each treatment option is beyond the scope of this introduction. More information can be obtained by visiting the various sections of the LIBRARY on our website, viewing a video on current treatment options from a recent IWMF Educational Forum, and linking to the patient-friendly publications at the end of this page. Currently available treatment options may include one or more of the following:
- supportive therapy such as transfusions or growth factors to boost red blood cells, white blood cells, and platelets;
- surgical or other procedures, including splenectomy (surgical removal of the spleen), plasmapheresis to remove IgM, targeted radiation to reduce the size of lymph nodes, and stem cell transplantation;
- chemotherapy with alkylating agents such as chlorambucil, cyclophosphamide, and bendamustine or with nucleoside analogs such as fludarabine and cladribine;
- corticosteroids, including prednisone and dexamethasone;
- biologic therapy with monoclonal antibodies such as rituximab and ofatumumab;
- immunomodulatory drugs, including thalidomide and lenalidomide;
- proteasome inhibitors such as bortezomib and carfilzomib;
- targeted therapies to the B-cell signaling pathways, including ibrutinib and everolimus.
Treatment can usually be administered in an outpatient setting or at home and may be oral, by intramuscular or subcutaneous injection, or by intravenous therapy. Some treatments require that certain medications be taken the day before or the day of treatment in order to minimize associated side effects.
Additionally, in some cases, participation in a clinical trial can actually be the best or even the only option available - and should be seriously considered by the patient. More information regarding clinical trials and their significance to WM patients can be found here.
Laboratory tests are performed during treatment to check response to therapy and/or to monitor side effects related to it. Typical laboratory testing includes the Complete Blood Count (CBC), the Complete Metabolic Panel (CMP), and the IgM measurement by nephelometry and/or serum protein electrophoresis (SPEP). Special circumstances or conditions might require additional tests.
Treatment cycles may take several weeks to months, depending on the course of therapy chosen. It is not unusual to have a round of therapy and then wait a week or a month before another round of treatment, although some of the newer oral therapies require daily dosing instead. Hematologist-oncologists follow established protocols for treatment but may make adjustments depending on side effects or response to therapy.
The following publications are written for patients and discuss many of the treatment options available for WM.
- Treatment Options Guides and Fact Sheets - a series of short patient-friendly guides, published in several languages by the IWMF, that can be found in IWMF Publications.
NCCN Guidelines for Patients with WM, 2017. This is a recent addition to the NCCN’s extensive series of patient guides for a variety of cancers and has been endorsed by the IWMF. It is currently available only in English.