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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:
1) an alkylating agent (like chlorambucil or cyclophosphamide)
2) a nucleoside analog (like fludarabine or cladribine)
3) the monoclonal antibody, rituximab
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, and without another treatment (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 Revised: 01/02/2008
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