Most experts recommend that treatment for Waldenstrom macroglobulinemia should wait until the disease is causing problems. This lets patients avoid the side effects of chemotherapy until they really need these medicines. In fact, studies suggest that patients who start chemotherapy as soon as they are diagnosed do not live any longer than those who delay treatment until their WM is causing problems. Doctors agree that hyperviscosity syndrome is a reason for immediate treatment, as it can be fatal. Other reasons for starting treatment include problems from amyloidosis and symptoms from cryoglobulinemia, as well as anemia (low red blood cells), kidney problems, heart problems, nerve damage, or any severe symptom from the WM.

Once a decision has been made to start chemotherapy, there are several options. Current guidelines suggest starting treatment with one of the following:

• an alkylating agent (like chlorambucil or cyclophosphamide)
• a nucleoside analog (like fludarabine or cladribine),
• the monoclonal antibody, rituximab
• the immunomodulating drug thalidomide plus dexamethasone
• bortezomib

A corticosteroid such as dexamethasone or prednisone is often used along with one of these drugs. These drugs are given in a variety of combinations and schedules depending on the individual situation. Some doctors like to use a combination of drugs at the beginning of treatment. In general, rituximab is not usually given by itself when the IgM level is very high, because it can make the IgM level temporarily go up even higher. Also, many experts recommend avoiding the nucleoside analogs if a stem cell transplant is planned for the future. Patients should ask their oncologists about schedules that are effective and convenient to them.

Many different chemotherapy combinations have been used in WM, including:

• chlorambucil with prednisone
• cyclophosphamide, adriamycin, vincristine, prednisone, and rituximab (called CHOP-R)
• cyclophosphamide, dexamethasone, and rituximab
• cyclophosphamide and rituximab
• fludarabine and cyclophosphamide
• fludarabine, cyclophosphamide, and rituximab

If a patient has hyperviscosity syndrome or if levels of the abnormal IgM protein are very high, the doctor may recommend starting plasmapheresis before chemotherapy. This procedure removes some of the abnormal IgM from the bloodstream and will lower IgM levels temporarily. Plasmapheresis does not affect the lymphoma cells that make the protein, so without a treatment to kill the lymphoma cells (like chemotherapy or immunotherapy) the IgM protein will just go back up. Plasmapheresis is usually given to help the patient until the chemotherapy has a chance to work.

Last Medical Review: 08/04/2009
Last Revised: 08/04/2009