Chemotherapy. Chemotherapy is the use of drugs to kill cancer cells, usually by stopping the cancer cells’ ability to grow and divide. Systemic chemotherapy is delivered through the bloodstream to reach cancer cells throughout the body. Chemotherapy is given by a medical oncologist or hematologist, doctors who specialize in treating this type of cancer with medication. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a set period of time. A patient may receive one drug at a time or combinations of different drugs at the same time.
The commonly used drugs for people with Waldenström’s macroglobulinemia include chlorambucil (Leukeran), cyclophosphamide (Neosar), fludarabine (Fludara), and cladribine (Leustatin).
The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished.
Getting Care for Symptoms and Side Effects. Cancer and its treatment often cause side effects. In addition to treatment to slow, stop, or eliminate the cancer, an important part of cancer care is relieving a person’s symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs. Palliative care can help a person at any stage of illness. People often receive treatment for the cancer and treatment to ease side effects at the same time. In fact, patients who receive both often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.
Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, and other therapies. You may also receive palliative treatments similar to those meant to eliminate the cancer, such as chemotherapy, surgery, and radiation therapy. Talk with your doctor about the goals of each treatment in the treatment plan. Before treatment begins, talk with your health care team about the possible side effects of your specific treatment plan and supportive care options. And during and after treatment, be sure to tell your doctor or another health care team member if you are experiencing a problem so it is addressed as quickly as possible.
Plasma Exchange. Plasma exchange (also called plasmapheresis) is used to reduce the thickness of the blood. It is done to help relieve the symptoms of hyperviscosity in people with Waldenström’s macroglobulinemia. During this procedure, blood is taken from a vein, and plasma (the liquid part of the blood) is removed from the body after it is separated from the red and white blood cells. The blood cells are then mixed with a plasma substitute, usually an albumin solution, and returned to the patient. This procedure is usually done using a blood cell separator.
Recurrent Waldenström’s Macroglobulinemia. A remission is when cancer cannot be detected in the body and there are no symptoms. This may also be called “no evidence of disease” or NED. A remission can be temporary or permanent. This uncertainty leads to many survivors feeling worried or anxious that the cancer will come back. While many remissions are permanent, it’s important to talk with your doctor about the possibility of the cancer returning. Understanding the risk of recurrence and the treatment options may help you feel more prepared if the cancer does return. If the cancer does return after the original treatment, it is called recurrent cancer. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence).
When this occurs, a cycle of testing will begin again to learn as much as possible about the recurrence, including whether your prognosis has changed. After testing is done, you and your doctor will talk about your treatment options. Often the treatment plan will include the therapies described above (such as chemotherapy, targeted therapy, and bone marrow/stem cell transplantation) but they may be used in a different combination or given at a different pace. Your doctor may also suggest clinical trials that are studying new ways to treat this type of recurrent cancer. People with recurrent cancer often experience emotions such as disbelief or fear. Patients are encouraged to talk with their health care team about these feelings and ask about support services to help them cope.
Refractory Waldenström’s Macroglobulinemia. If treatment does not stop or eliminate the disease, this is called refractory Waldenström’s macroglobulinemia. Patients with this diagnosis are encouraged to talk with doctors who are experienced in treating this stage of cancer, because there can be different opinions about the best treatment plan. Learn more about seeking a second opinion before starting treatment, so you are comfortable with the treatment plan chosen.
Your health care team may recommend a treatment plan that includes a combination of different chemotherapy drugs. Supportive care will also be important to help relieve symptoms and side effects. For most patients, this diagnosis is very stressful and, at times, difficult to bear. Patients and their families are encouraged to talk about the way they are feeling with doctors, nurses, social workers, or other members of the health care team. It may also be helpful to talk with other patients, including through a support group.
Stem Cell Transplantation/Bone Marrow Transplantation. Stem cell transplantation is a medical procedure in which bone marrow that contains cancer is replaced by highly specialized cells, called hematopoietic stem cells, that develop into healthy bone marrow. Hematopoietic stem cells are found both in the bloodstream and in the bone marrow. Today, this procedure is more commonly called a stem cell transplant, rather than bone marrow transplant, because it is the stem cells in the blood that are typically being transplanted, not the actual bone marrow tissue.
However, transplantation is not used often for people with Waldenström’s macroglobulinemia because most patients are older, the treatment does not work equally well for each patient, and there are serious risks with this treatment.
Targeted Therapy. Targeted therapy is a treatment that targets the cancer’s specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. This type of treatment blocks the growth and spread of cancer cells while limiting damage to normal cells. Recent studies show that not all tumors have the same targets. To find the most effective treatment, your doctor may run tests to identify the genes, proteins, and other factors in your tumor. As a result, doctors can better match each patient with the most effective treatment whenever possible. In addition, many research studies are taking place now to find out more about specific molecular targets and new treatments directed at them.
A monoclonal antibody is a type of targeted therapy. It is directed against a specific protein in the cancer cells and it does not affect cells that do not have that protein. Rituximab (Rituxan) is a monoclonal antibody used to treat many different types of B-cell lymphoma. Rituximab works by targeting a cell-surface molecule called CD20. When the antibody attaches to this antigen, some lymphoma cells die and others appear to become more susceptible to chemotherapy. Rituximab can be used either alone or in combination with chemotherapy for people with Waldenström’s macroglobulinemia.
Although it is quite effective by itself, there is increasing evidence that, for people with most types of B-cell non-Hodgkin lymphoma, rituximab works better when combined with chemotherapy. Talk with your doctor about possible side effects for rituximab and how they can be managed.
Watchful Waiting. Some patients with Waldenström’s macroglobulinemia may not need immediate treatment if they are otherwise healthy and the disease is not causing any symptoms or problems. In these situations, patients are closely monitored, and active treatment begins if symptoms develop or the IgM level increases. This is called watchful waiting, watch-and-wait, or active surveillance. There is very good evidence that, in some patients with low-grade lymphoma, watchful waiting does not affect the chances of survival as long as the patient receives regular and careful follow-up.
If Treatment Fails
Recovery from cancer is not always possible. If treatment is not successful, the disease may be called advanced or terminal cancer. This diagnosis is stressful, and this is difficult to discuss for many people. However, it is important to have open and honest conversations with your doctor and health care team to express your feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families. Making sure a person is physically comfortable and free from pain is extremely important.
Patients who have advanced cancer and who are expected to live less than six months may want to consider a type of palliative care called hospice care. Hospice care is designed to provide the best possible quality of life for people who are near the end of life. You and your family are encouraged to think about where you would be most comfortable: at home, in the hospital, or in a hospice environment. Nursing care and special equipment can make staying at home a workable alternative for many families.