View All Pages

Waldenstrom’s Macroglobulinemia - Introduction

Approved by the Cancer.Net Editorial Board, 12/2018

ON THIS PAGE: You will find some basic information about Waldenstrom's macrobulinemia and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Waldenstrom’s Macroglobulinemia. Use the menu to see other pages. Think of that menu as a roadmap for this complete guide.

Waldenstrom’s macroglobulinemia is a type of non-Hodgkin lymphoma (NHL) that affects small lymphocytes, which are white blood cells.

About lymphoma and the lymph system

NHL is a term that refers to many types of cancer of the lymphatic system, which can have different symptoms and signs, physical findings, and treatment options.

The lymphatic system is made up of thin tubes that branch out to all parts of the body and helps fight infection. The lymphatic system carries lymph, a colorless fluid containing lymphocytes. Lymphocytes fight germs in the body. B-lymphocytes, also called B cells, make antibodies to fight bacteria. T-lymphocytes, or T cells, kill viruses and foreign cells and trigger the B cells to make antibodies.

Groups of bean-shaped organs called lymph nodes are located throughout the body at different sites in the lymphatic system. Lymph nodes are found in clusters in the abdomen, groin, pelvis, underarms, and neck. Other parts of the lymphatic system include the spleen, which makes lymphocytes and filters blood; the thymus, an organ under the breastbone; and the tonsils, located in the throat.

About Waldenstrom’s macroglobulinemia

Lymphoma begins when healthy cells in the lymphatic system change and grow out of control, which may form a tumor.

In Waldenstrom’s macroglobulinemia, the lymphocytes are similar in shape to plasma cells. Plasma cells make antibodies, which are specialized proteins that help make viruses and bacteria harmless to the body. One type of antibody made by plasma cells is called immunoglobulin M (IgM). Another name for Waldenstrom’s macroglobulinemia is lymphoplasmacytic lymphoma. It is classified as an indolent, or low-grade, type of lymphoma. Learn more about the subtypes of NHL.

Because lymphatic tissue is found in so many parts of the body, Waldenstrom’s macroglobulinemia can start almost anywhere and may spread to almost any organ in the body. When people are first diagnosed with the disease, it has usually already spread to the blood and bone marrow. It may also eventually affect the lymph nodes, liver, or spleen, and rarely the stomach, intestines, lungs, skin, or thyroid gland.

The term “Waldenstrom’s macroglobulinemia” usually is meant for those with high levels of IgM in the blood and who have lymphoplasmacytic lymphoma. Because of the similarity of Waldenstrom’s macroglobulinemia with some B-cell lymphomas or multiple myeloma, treatment can often be similar for these patients. You should talk with your health care team about the specific diagnosis and what treatment plan is best for you.

Looking for More of an Introduction?

If you would like more of an introduction, explore these related items. Please note that these links will take you to other sections on Cancer.Net:

  • ASCO Answers Fact Sheet: Read a 1-page fact sheet that offers an introduction to non-Hodgkin lymphoma. This fact sheet is available as a PDF, so it is easy to print out.

The next section in this guide is Statistics. It helps explain the number of people who are diagnosed with Waldenstrom's macroglobulinemia and general survival rates. Use the menu to choose a different section to read in this guide.

Waldenstrom’s Macroglobulinemia - Statistics

Approved by the Cancer.Net Editorial Board, 01/2019

ON THIS PAGE: You will find information about the number of people who are diagnosed with Waldenstrom’s macroglobulinemia each year. You will also read general information on surviving the disease. Remember, survival rates depend on several factors. Use the menu to see other pages.

Waldenstrom’s macroglobulinemia is uncommon. Each year, an estimated 1,000 to 1,500 adults in the United States are diagnosed with the disease. The chance of developing Waldenstrom’s macroglobulinemia increases as people age.

The 5-year survival rate tells you what percent of people live at least 5 years after the cancer is found. Percent means how many out of 100. The 5-year survival rate for people with Waldenstrom’s macroglobulinemia is about 78%. However, it is important to note that survival rates vary based on a number of individual factors, including the patient’s age, how advanced the disease is at the time of diagnosis, and whether the patient has other medical problems.

It is important to remember that statistics on the survival rates for people with Waldenstrom’s macroglobulinemia are an estimate. The estimate comes from annual data based on the number of people with this cancer in the United States. Also, experts measure the survival statistics every 5 years. So the estimate may not show the results of better diagnosis or treatment available for less than 5 years. Talk with your doctor if you have any questions about this information. Learn more about understanding statistics.

Statistics adapted from the American Cancer Society (January 2019).

The next section in this guide is Risk Factors. It explains what factors may increase the chance of developing Waldenstrom’s macroglobulinemia. Use the menu to choose a different section to read in this guide.

Waldenstrom’s Macroglobulinemia - Risk Factors

Approved by the Cancer.Net Editorial Board, 12/2018

ON THIS PAGE: You will find out more about the factors that increase the chance of developing Waldenstrom's macroglobulinemia. Use the menu to see other pages.

A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors often influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do. Knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices.

The following factors may raise a person’s risk of developing Waldenstrom’s macroglobulinemia:

  • Age. The risk of Waldenstrom’s macroglobulinemia increases with age. It occurs most commonly in people older than 60.

  • Gender. Men are more likely to develop Waldenstrom’s macroglobulinemia than women.

  • Race. White people are more likely to develop Waldenstrom’s macroglobulinemia than black people.

  • Monoclonal gammopathy of undetermined significance (MGUS). MGUS is a buildup of monoclonal antibodies produced by abnormal plasma cells. MGUS does not generally cause symptoms or many health problems, although the abnormal antibody can occasionally bind to nerves and cause muscle weakness, tingling, or numbness. In rare cases, the monoclonal antibody can lead to a decrease in kidney function. But about 20% of people with MGUS will develop non-Hodgkin lymphoma or multiple myeloma within 20 years.

The next section in this guide is Symptoms and Signs. It explains what body changes or medical problems Waldenstrom's macroglobulinemia can cause. Use the menu to choose a different section to read in this guide.

Waldenstrom's Macroglobulinemia - Symptoms and Signs

Approved by the Cancer.Net Editorial Board, 12/2018

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. Use the menu to see other pages.

People with Waldenstrom’s macroglobulinemia may experience the following symptoms or signs. Sometimes, people with Waldenstrom’s macroglobulinemia do not have any of these changes. Or, the cause of a symptom may be a different medical condition that is not cancer.

  • Fatigue

  • Unexplained weight loss

  • Enlarged lymph nodes or spleen

  • Numbness, weakness or other nervous system problems, pain in the hands or feet, sometimes called peripheral neuropathy

  • Abdominal swelling and diarrhea

  • Weakness and shortness of breath

  • Infections

  • Raised pink or flesh-colored lesions on the skin

  • Changes in the color of the fingertips when exposed to cold

  • Changes in vision, which may include blurry vision or “double” vision

Certain symptoms, called B symptoms, may signal a more aggressive cancer. Doctors may refer to either “A” or “B” when describing the lymphoma.

A means that a person has not experienced B symptoms, listed below.

B means that a person has experienced the following symptoms:

  • Unexplained weight loss

  • Unexplained fever

  • Heavy sweating, especially at night, which may drench one’s nightclothes or sheets on the bed.

  • Severe and/or extensive skin itchiness

Symptoms of hyperviscosity

IgM proteins are large molecules, and when they accumulate in the blood in high levels, the blood can become viscous or thick. This slows down the flow of blood to different parts of the body. Symptoms of hyperviscosity include:

  • Vision problems, especially blurred or double vision

  • Confusion

  • Dizziness

  • Loss of coordination

  • Headaches

  • Nosebleeds or bleeding gums

  • Fatigue

  • Shortness of breath

If you are concerned about any changes you experience, please talk with your doctor. Your doctor will ask how long and how often you have been experiencing the symptom(s), in addition to other questions. This is to help figure out the cause of the problem, called a diagnosis.

If cancer is diagnosed, relieving symptoms remains an important part of cancer care and treatment. This may be called palliative care or supportive care. Be sure to talk with your health care team about the symptoms you experience, including any new symptoms or a change in symptoms.

The next section in this guide is Diagnosis. It explains what tests may be needed to learn more about the cause of the symptoms. Use the menu to choose a different section to read in this guide.

Waldenstrom's Macroglobulinemia - Diagnosis

Approved by the Cancer.Net Editorial Board, 12/2018

ON THIS PAGE: You will find a list of common tests, procedures, and scans that doctors use to find the cause of a medical problem. Use the menu to see other pages.

Doctors use many tests to find, or diagnose, cancer. They also do tests to learn if cancer has spread to another part of the body from where it started. If this happens, it is called metastasis. For example, imaging tests can show if the cancer has spread. Imaging tests show pictures of the inside of the body. Doctors may also do tests to learn which treatments could work best.

For most types of cancer, a biopsy is the only sure way for the doctor to know if an area of the body has cancer. In a biopsy, the doctor takes a small sample of tissue for testing in a laboratory. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis.

This list describes options for diagnosing this type of cancer. Not all tests listed below will be used for every person. Your doctor may consider these factors when choosing a diagnostic test:

  • The type of cancer suspected

  • Your signs and symptoms

  • Your age and general health

  • The results of earlier medical tests

In addition to a physical examination, the following tests may be used to diagnose Waldenstrom’s macroglobulinemia:

Blood tests. Blood tests may include a CBC (complete blood count) with a differential (classification of the types of white cells) and an examination of the blood with a microscope. Blood tests can help your doctor diagnose Waldenstrom’s macroglobulinemia. An increase in IgM can be found by protein electrophoresis, which is a method of separating proteins in the blood with an electric field.

Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. A pathologist then analyzes the sample(s). A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease. The type of biopsy performed depends on the location of the cancer.

The most common type of biopsy for Waldenstrom’s macroglobulinemia is either a bone marrow biopsy or a biopsy of the lymph nodes in the neck, under the arms, or in the groin. A biopsy may also be taken from the chest or abdomen while using a computed tomography (CT or CAT scan) to guide the doctor. A biopsy can also be taken from the stomach or intestine during an endoscopy, a test that allows the doctor to see inside the body using a thin, lighted, flexible tube called an endoscope. More information on bone marrow biopsy and a CT scan is below.

Bone marrow aspiration and biopsy. Lymphoma often spreads to the bone marrow, the spongy material in the center of bones where blood cells are produced. A sample of the bone marrow can be important to diagnose Waldenstrom’s macroglobulinemia, and it can help find out if the cancer has spread.

These 2 procedures are similar and often done at the same time to examine the bone marrow. Bone marrow has both a solid and a liquid part. A bone marrow aspiration removes a sample of the fluid with a needle. A bone marrow biopsy is the removal of a small amount of solid tissue using a different needle.

A pathologist then analyzes the sample(s). A common site for a bone marrow aspiration and biopsy is the pelvic bone, which is located in the lower back by the hip. The skin in that area is usually numbed with medication beforehand. Other types of anesthesia (medication to block the awareness of pain) may also be used.

Tests on the biopsy sample may be done to examine proteins on the tumor cells in order to distinguish Waldenstrom's macroglobulinemia from other types of B-cell lymphomas. These types of tests are called immunohistochemistry and/or flow cytometry.

Molecular testing of the tumor. Your doctor may recommend running laboratory tests on a tissue sample and/or bone marrow sample to identify specific genes, proteins, and other factors unique to the tumor. Molecular tests can also help distinguish Waldenstrom's macroglobulinemia from other B-cell lymphomas. See the Types of Treatment section for more information. Doctors look for a mutation in the MYD88 gene. This mutation is found in Waldenstrom’s macroglobulinemia for most patients, but some patients whose tissue sample has this mutation might have a different lymphoma or other disease.

Computed tomography (CT or CAT) scan. A CT scan takes pictures of the inside of the body using x-rays taken from different angles. A computer combines these images into a detailed, 3-dimensional view that shows any abnormalities or tumors. A CT scan can be used to measure the tumor’s size. Sometimes, special dyes, called contrast mediums, are given before the scan to provide better detail on the image. Dyes are injected into a patient’s vein or given as a pill or liquid to swallow.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. MRI can be used to measure the tumor’s size. A special dye called a contrast medium is given before the scan to create a clearer picture. This dye is injected into a patient’s vein.

Positron emission tomography (PET) or PET-CT scan. A PET scan is usually combined with a CT scan (see above), called a PET-CT scan. However, you may hear your doctor refer to this procedure just as a PET scan. A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive sugar substance is injected into the patient’s body. This sugar substance is taken up by cells that use the most energy. Because cancer tends to use energy actively, it absorbs more of the radioactive substance. A scanner then detects this substance to produce images of the inside of the body.

Fundascopic evaluation. This exam of the eye is done by an ophthalmologist. An ophthalmologist is a medical doctor who specializes in eye care. This test looks at the blood vessels of the eye.

Urine tests. The doctor tests a urine sample to check for abnormal proteins in the body.

After diagnostic tests are done, your doctor will review all of the results with you. If the diagnosis is cancer, these results also help the doctor describe the extent of spread of the cancer. This is called staging.

The next section in this guide is Stages. It explains the system doctors use to describe the extent of Waldenstrom's macroglobulinemia. Use the menu to choose a different section to read in this guide.

Waldenstrom's Macroglobulinemia - Stages

Approved by the Cancer.Net Editorial Board, 12/2018

ON THIS PAGE: You will learn about how doctors describe a cancer’s growth or spread. This is called the stage. Use the menu to see other pages.

Staging is a way of describing where the cancer is located, if or where it has spread, and whether it is affecting other parts of the body. Doctors use diagnostic tests to find out the cancer's stage, so staging may not be complete until all the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis, which is the chance of recovery. There are different stage descriptions for different types of cancer.

There is no standard system for staging Waldenstrom’s macroglobulinemia. Instead, doctors may look at several factors to determine a prognosis, such as:

  • the severity of a patient’s anemia

  • a low level of red blood cells

  • the amount of IgM in the blood

  • the amount of a protein in the blood called beta-2-microglobulin

People with low beta-2-microglobulin levels and normal hemoglobin—the protein that carries oxygen in a red blood cell—typically have a better prognosis.

Information about the cancer’s factors will help the doctor recommend a specific treatment plan. The next section in this guide is Types of Treatment. Use the menu to choose a different section to read in this guide.

Waldenstrom's Macroglobulinemia - Treatment Options

Approved by the Cancer.Net Editorial Board, 12/2018

ON THIS PAGE: You will learn about the different types of treatments doctors use for people with Waldenstrom's macroglobulinemia. Use the menu to see other pages.

This section explains the types of treatments that are the standard of care for Waldenstrom's macroglobulinemia. “Standard of care” means the best treatments known. When making treatment plan decisions, you are encouraged to consider clinical trials as an option. A clinical trial is a research study that tests a new approach to treatment. Doctors want to learn whether the new treatment is safe, effective, and possibly better than the standard treatment. Clinical trials can test a new drug, a new combination of standard treatments, or new doses of standard drugs or other treatments. Clinical trials are an option to consider for treatment and care for all stages of cancer. Your doctor can help you consider all your treatment options. To learn more about clinical trials, see the Clinical Trials and Latest Research sections.

Treatment overview

In cancer care, different types of doctors often work together to create a patient’s overall treatment plan that combines different types of treatments. This is called a multidisciplinary team. Cancer care teams include a variety of other health care professionals, such as physician assistants, oncology nurses, social workers, pharmacists, counselors, dietitians, and others.

Descriptions of the common types of treatments used for Waldenstrom’s macroglobulinemia are listed below. Your care plan also includes treatment for symptoms and side effects, an important part of cancer care.

Treatment options and recommendations depend on several factors, including the type and stage of cancer, possible side effects, and the patient’s preferences and overall health. Take time to learn about all of your treatment options and be sure to ask questions about things that are unclear. Also, talk about the goals of each treatment with your doctor and what you can expect while receiving the treatment. Treatment goals for Waldenstrom’s is to treat the disease and/or to relieve the symptoms for as many years as possible. Talk with your health care team to understand the goals of each treatment. Learn more about making treatment decisions.

Watchful waiting

Some people with Waldenstrom’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 people with low-grade lymphoma, watchful waiting does not affect the chances of survival as long as the patient receives regular and careful follow-up. Sometimes, treatment can be delayed for many months or even years.

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 Waldenstrom’s macroglobulinemia from the elevated IgM level. 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.

Therapies using medication

Systemic therapy is the use of medication to destroy cancer cells. This type of medication is given through the bloodstream to reach cancer cells throughout the body. Systemic therapies are generally prescribed by a medical oncologist, a doctor who specializes in treating cancer with medication.

Common ways to give systemic therapies include an intravenous (IV) tube placed into a vein using a needle or in a pill or capsule that is swallowed (orally).

The types of systemic therapies used for Waldenstrom's macroglobulinemia include:

  • Chemotherapy

  • Targeted therapy

Each of these types of therapies is discussed below in more detail. A person may receive only 1 type of systemic therapy at a time or a combination of systemic therapies given at the same time. They can also be given as part of a treatment plan that includes surgery and/or radiation therapy.

The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions by using searchable drug databases.

Chemotherapy

Chemotherapy is the use of drugs to destroy cancer cells, usually by blocking the cancer cells’ ability to grow and divide.

A chemotherapy regimen, or schedule, usually consists of a specific number of cycles given over a set period of time. A patient may receive 1 drug at a time or a combination of different drugs given at the same time. Common drugs for Waldenstrom’s macroglobulinemia include chlorambucil (Leukeran), cladribine (Leustatin), cyclophosphamide (Neosar), and fludarabine (Fludara). Bendamustine (Treanda) is a chemotherapy treatment that can be used to treat Waldenstrom’s macroglobulinemia when it is first diagnosed or when it returns.

The side effects of chemotherapy depend on the individual, the type of drug 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 after treatment is finished.

Learn more about the basics of chemotherapy.

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 healthy cells.

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. This helps doctors better match each patient with the most effective treatment whenever possible. In addition, research studies continue to find out more about specific molecular targets and new treatments directed at them. Learn more about the basics of targeted treatments.

Targeted therapy for Waldenstrom's macroglobulinemia includes monoclonal antibodies. 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 Waldenstrom’s macroglobulinemia. Although it is 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. Other anti-CD20 antibodies include obinutuzumab (Gazyva) ofatumomab (Arzerra), and rituximab-abbs (Truximal).

Another targeted therapy, called ibrutinib (Imbruvica), works to target BTK proteins in cancerous B-cells. Ibrutinib was approved in 2015 by the Food and Drug Administration (FDA) as a treatment for Waldenstrom’s macroglobulinemia, after earlier approvals for 2 other types of blood-related cancers. Bortezomib (Velcade) is another type of targeted therapy called a proteasome inhibitor, targeting specific enzymes called proteasomes that digest proteins in the cells.

Talk with your doctor about possible side effects for each targeted therapy and how they can be managed.

Bone marrow transplantation/stem cell transplantation

A bone marrow transplant 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.

Transplantation is not used often for people with Waldenstrom’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. Side effects depend on the type of transplant, your general health, and other factors. Learn more about the basics of bone marrow and stem cell transplantation.

Care for symptoms and side effects

Cancer and its treatment often cause side effects. In addition to treatments intended 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 is any treatment that focuses on reducing symptoms, improving quality of life, and supporting patients and their families. Any person, regardless of age or type and stage of cancer, may receive palliative care. It works best when palliative care is started as early as needed in the cancer treatment process. People often receive treatment for the cancer at the same time that they receive treatment to ease side effects. In fact, people who receive both at the same time 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, emotional support and other therapies. You may also receive palliative treatments similar to those meant to eliminate the cancer, such as chemotherapy, surgery, or 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 the specific treatment plan and palliative care options. During and after treatment, be sure to tell your doctor or another health care team member if you are experiencing a problem so it can be addressed as quickly as possible. Learn more about palliative care.

Refractory Waldenstrom’s macroglobulinemia

If treatment does not stop or eliminate the disease, this is called refractory Waldenstrom’s macroglobulinemia. If this happens, it is a good idea to talk with doctors who have experience in treating it. Doctors can have different opinions about the best standard treatment plan. Clinical trials might also be an option. Learn more about getting a second opinion before starting treatment, so you are comfortable with your treatment plan chosen.

Your treatment plan may include a combination of different chemotherapy drugs. Palliative care will also be important to help relieve symptoms and side effects.

For most people, this diagnosis is very stressful and, at times, difficult to bear. You and your family are encouraged to talk about how you 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.

Remission and the chance of recurrence

A remission is when cancer cannot be detected in the body and there are no symptoms. This may also be called having “no evidence of disease” or NED.

A remission may be temporary or permanent. This uncertainty causes many people to worry that the cancer will come back. While many remissions are permanent, it is important to talk with your doctor about the possibility of the cancer returning. Understanding your risk of recurrence and the treatment options may help you feel more prepared if the cancer does return. Learn more about coping with the fear of recurrence.

If Waldenstrom’s macroglobulinemia returns after the original treatment, it is called recurrent disease. When this occurs, a new cycle of testing will begin again to learn as much as possible about the recurrence. After this testing is done, you and your doctor will talk about the treatment options. Often the treatment plan will include the treatments described above, such as chemotherapy, targeted therapy, and rarely, bone marrow/stem cell transplantation. But they may be used in a different combination or given at a different pace. Your doctor may suggest clinical trials that are studying new ways to treat this type of recurrent cancer. There are currently several new drugs and combinations of different therapies being researched in clinical trials. Whichever treatment plan you choose, palliative care will be important for relieving symptoms and side effects.

People with recurrent cancer often experience emotions such as disbelief or fear. You are encouraged to talk with the health care team about these feelings and ask about support services to help you cope. Learn more about dealing with cancer recurrence.

If treatment does not work

Recovery from cancer is not always possible. If the cancer cannot be cured or controlled, the disease may be called advanced or terminal.

This diagnosis is stressful, and for many people, advanced cancer is difficult to discuss. However, it is important to have open and honest conversations with your 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.

People who have advanced cancer and who are expected to live less than 6 months may want to consider 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 talk with the health care team about hospice care options, which include hospice care at home, a special hospice center, or other health care locations. Nursing care and special equipment can make staying at home a workable option for many families. Learn more about advanced cancer care planning.

After the death of a loved one, many people need support to help them cope with the loss. Learn more about grief and loss.

The next section in this guide is About Clinical Trials. It offers more information about research studies that are focused on finding better ways to care for people with cancer. Use the menu to choose a different section to read in this guide.

Waldenstrom's Macroglobulinemia - About Clinical Trials

Approved by the Cancer.Net Editorial Board, 12/2018

ON THIS PAGE: You will learn more about clinical trials, which are the main way that new medical approaches are tested to see how well they work. Use the menu to see other pages.

What are clinical trials?

Doctors and scientists are always looking for better ways to care for people with Waldenstrom’s macroglobulinemia. To make scientific advances, doctors create research studies involving volunteers, called clinical trials. In fact, every drug that is now approved by the U.S. Food and Drug Administration (FDA) was tested in clinical trials.

Many clinical trials focus on new treatments. Researchers want to learn if a new treatment is safe, effective, and possibly better than the treatment doctors use now. These types of studies evaluate new drugs, different combinations of existing treatments, new approaches to chemotherapy or surgery, and new methods of treatment. People who participate in clinical trials can be some of the first to get a treatment before it is available to the public. However, there are some risks with a clinical trial, including possible side effects and that the new treatment may not work. People are encouraged to talk with their health care team about the pros and cons of joining a specific study.

Some clinical trials study new ways to relieve symptoms and side effects during treatment. Others study ways to manage the late effects that may happen a long time after treatment. Talk with your doctor about clinical trials for symptoms and side effects. There are also clinical trials studying ways to prevent cancer.

Deciding to join a clinical trial

People decide to participate in clinical trials for many reasons. For some people, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other people volunteer for clinical trials because they know that these studies are a way to contribute to the progress in treating Waldenstrom’s macroglobulinemia. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with Waldenstrom’s macroglobulinemia.

Insurance coverage of clinical trials costs differs by location and by study. In some programs, some of the expenses from participating in the clinical trial are reimbursed. In others, they are not. It is important to talk with the research team and your insurance company first to learn if and how your treatment in a clinical trial will be covered. Learn more about health insurance coverage of clinical trials.

Sometimes people have concerns that, in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” Placebos are usually combined with standard treatment in most cancer clinical trials. When a placebo is used in a study, it is done with the full knowledge of the participants. Find out more about placebos in cancer clinical trials.

Patient safety and informed consent

To join a clinical trial, people must participate in a process known as informed consent. During informed consent, the doctor should:

  • Describe all of the treatment options so that the person understands the standard treatments, and how the new treatment differs from the standard treatment.

  • List all of the risks of the new treatment, which may or may not be different than the risks of standard treatment.

  • Explain what will be required of each person in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment.

Clinical trials also have certain rules called “eligibility criteria” that help structure the research and keep patients safe. You and the research team will carefully review these criteria together.

People who participate in a clinical trial may stop participating at any time for any personal or medical reason. This may include that the new treatment is not working or there are serious side effects. Clinical trials are also closely monitored by experts who watch for any problems with each study. It is important that people participating in a clinical trial talk with their doctor and researchers about who will be providing their treatment and care during the clinical trial, after the clinical trial ends, and/or if they choose to leave the clinical trial before it ends.

Finding a clinical trial

Research through clinical trials is ongoing for all types of cancer. For specific topics being studied for Waldenstrom’s macroglobulinemia, learn more in the Latest Research section.

Cancer.Net offers a lot of information about cancer clinical trials in other areas of the website, including a complete section on clinical trials and places to search for clinical trials for a specific type of cancer.

In addition, this website offers free access to a video-based educational program about cancer clinical trials, located outside of this guide.

null

The next section in this guide is Latest Research. It explains areas of scientific research currently going on for Waldenstrom's macroglobulinemia. Use the menu to choose a different section to read in this guide.

Waldenstrom's Macroglobulinemia - Latest Research

Approved by the Cancer.Net Editorial Board, 12/2018

ON THIS PAGE: You will read about the scientific research being done now to learn more about this type of cancer and how to treat it. Use the menu to see other pages.

Doctors are working to learn more about Waldenstrom’s macroglobulinemia, ways to prevent it, how to best treat it, and how to provide the best care to people diagnosed with Waldenstrom's macroglobulinemia. The following areas of research may include new options for patients through clinical trials. Always talk with your doctor about the best diagnostic and treatment options for you.

New treatments and targeted therapy. As explained in the Treatment Options section, targeted therapy can block tumor growth in different ways. Other drugs that target how B-cells develop are also being evaluated for patients with Waldenstrom’s macroglobulinemia. Several drugs that inhibit PI3 kinase are available and being evaluated for a variety of B-cell cancers, including Waldenstrom’s macroglobulinemia. The drugs everolimus (Afinitor) and temsirolimus (Torisel), as well as drugs called histone deacetylase (HDAC) inhibitors, such as panobinostat (Farydak), are also being looked at as treatments for Waldenstrom’s macroglobulinemia.

Drug combinations. Drugs are being used in combination to treat Waldenstrom’s macroglobulinemia:

  • Monoclonal antibodies, such as rituximab, which are described in the Types of Treatment section, are being used along with chemotherapy as a way to eliminate more cancer cells.

    The drug fludarabine combined with cyclophosphamide is sometimes used to treat patients with advanced and/or symptomatic Waldenstrom’s macroglobulinemia, as is thalidomide (Synovir, Thalomid) or lenalidomide (Revlimid) plus rituximab. Another combination of drugs used is called DTPACE. This contains the following drugs:

    • Cisplatin (Platinol)

    • Dexamethasone (multiple brand names)

    • Doxorubicin (Adriamycin)

    • Cyclophosphamide

    • Etoposide (Toposar, VePesid)

  • Bortezomib, dexamethasone, and rituximab recently have been shown to be an active combination to treat Waldenstrom’s macroglobulinemia.

Palliative care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current Waldenstrom’s macroglobulinemia treatments to improve comfort and quality of life for patients.

Looking for More about the Latest Research?

If you would like additional information about the latest areas of research regarding Waldenstrom’s macroglobulinemia, explore these related items that take you outside of this guide:

The next section in this guide is Coping with Treatment. It offers some guidance in how to cope with the physical, emotional, and social changes that cancer and its treatment can bring. Use the menu to choose a different section to read in this guide.

Waldenstrom's Macroglobulinemia - Coping with Treatment

Approved by the Cancer.Net Editorial Board, 12/2018

ON THIS PAGE: You will learn more about coping with the physical, emotional, social, and financial effects of cancer and its treatment. This page includes several links outside of this guide to other sections of this website. Use the menu to see other pages.

Every cancer treatment can cause side effects or changes to your body and how you feel. For many reasons, people do not experience the same side effects even when they are given the same treatment for the same type of cancer. This can make it hard to predict how you will feel during treatment.

As you prepare to start cancer treatment, it is normal to fear treatment-related side effects. It may help to know that your health care team will work to prevent and relieve side effects. Doctors call this part of cancer treatment “palliative care” or "supportive care." It is an important part of your treatment plan, regardless of your age or the stage of disease.

Coping with physical side effects

Common physical side effects from each treatment option for Waldenstrom’s macroglobulinemia are listed in the Types of Treatment section. Learn more about side effects of cancer and its treatment, along with ways to prevent or control them. Changes to your physical health depend on several factors, including the cancer’s stage, the length and dose of treatment, and your general health.

Sometimes, physical side effects can last after treatment ends. Doctors call these long-term side effects. They call side effects that occur months or years after treatment late effects. Treating long-term side effects and late effects is an important part of survivorship care. Learn more by reading the Follow-up Care section of this guide or talking with your doctor.

Coping with emotional and social effects

You can have emotional and social effects as well as physical effects after a cancer diagnosis. This may include dealing with difficult emotions, such as sadness, anxiety, anger, or managing your stress level. Sometimes, patients have problems expressing how they feel to their loved ones, or people do not know what to say in response.

Patients and their families are encouraged to share their feelings with a member of their health care team. You can also find coping strategies for emotional and social effects in a separate section of this website. This section includes many resources for finding support and information to meet your needs.

Coping with financial effects

Cancer treatment can be expensive. It is often a big source of stress and anxiety for people with cancer and their families. In addition to treatment costs, many people find they have extra, unplanned expenses related to their care. For some people, the high cost of medical care stops them from following or completing their cancer treatment plan. This can put their health at risk and may lead to higher costs in the future. Patients and their families are encouraged to talk about financial concerns with a member of their health care team. Learn more about managing financial considerations, in a separate part of this website.

Caring for a loved one with cancer

Family members and friends often play an important role in taking care of a person with Waldenstrom’s macroglobulinemia. This is called being a caregiver. Caregivers can provide physical, practical, and emotional support to the patient, even if they live far away.

Caregivers may have a range of responsibilities on a daily or as-needed basis. Below are some of the responsibilities caregivers take care of:

  • Providing support and encouragement

  • Talking with the health care team

  • Giving medications

  • Helping manage symptoms and side effects

  • Coordinating medical appointments

  • Providing a ride to and from appointments

  • Assisting with meals

  • Helping with household chores

  • Handling insurance and billing issues

Learn more about caregiving.

Talking with your health care team about side effects

Before starting treatment, talk with your doctor about possible side effects. Ask:

  • Which side effects are most likely?

  • When are they likely to happen?

  • What can we do to prevent or relieve them?

Be sure to tell your health care team about any side effects that happen during treatment and afterward, too. Tell them even if you do not think the side effects are serious. This discussion should include physical, emotional, and social effects of cancer.

Also, ask how much care you may need at home and with daily tasks during and after treatment. This can help you make a caregiving plan.

The next section in this guide is Follow-up Care. It explains the importance of checkups after you finish cancer treatment. Use the menu to choose a different section to read in this guide.

Waldenstrom's Macroglobulinemia - Follow-Up Care

Approved by the Cancer.Net Editorial Board, 12/2018

ON THIS PAGE: You will read about your medical care after cancer treatment is completed and why this follow-up care is important use the menu.

Care for people diagnosed with cancer does not end when active treatment has finished. Your health care team will continue to check to make sure the cancer has not returned, manage any side effects, and monitor your overall health. This is called follow-up care.

Your follow-up care may include regular physical examinations, medical tests, or both. Doctors want to keep track of your recovery in the months and years ahead. This plan may include regular physical examinations and/or medical tests to monitor your recovery for the coming months and years. Typically, follow-up visits are most frequent in the first 3 years after treatment.

Several long-term side effects of cancer treatment may occur:

  • Heart damage in the form of a weakened heart muscle may occur in people who have received a higher dose of doxorubicin.

  • Infertility, which is the inability to have children, as well as premature, or early, menopause can occur in people who have received high-dose cyclophosphamide or other types of chemotherapy.

  • Secondary cancers, which are other cancers that develop because of the original cancer treatments, are more common in people who received some types of chemotherapy.

Talk with your doctor about possible long-term effects of specific treatments you received. Learn more about the importance of follow-up care.

Watching for recurrence

One goal of follow-up care is to check for a recurrence. Cancer recurs because small areas of cancer cells may remain undetected in the body. Over time, these cells may increase in number until they show up on test results or cause signs or symptoms. During follow-up care, a doctor familiar with your medical history can give you personalized information about your risk of recurrence. Your doctor will ask specific questions about your health. Some people may have blood tests or imaging tests done as part of regular follow-up care, but testing recommendations depend on several factors including the type and stage of cancer originally diagnosed and the types of treatment given. Your doctor will periodically check the IgM in your blood to check for recurrence, along with an examination for enlarged lymph nodes or spleen. Tests such as CT scans or PET-CT scans may also be recommended.

The anticipation before having a follow-up test or waiting for test results can add stress to you or a family member. This is sometimes called “scan-xiety.” Learn more about how to cope with this type of stress.

Managing long-term and late side effects

Most people expect to experience side effects when receiving treatment. However, it is often surprising to survivors that some side effects may linger beyond the treatment period. These are called long-term side effects. Other side effects called late effects may develop months or even years afterwards. Long-term and late effects can include both physical and emotional changes.

Talk with your doctor about your risk of developing such side effects based on the type of cancer, your individual treatment plan, and your overall health. If you had a treatment known to cause specific late effects, you may have certain physical examinations, scans, or blood tests to help find and manage them.

Keeping personal health records

You and your doctor should work together to develop a personalized follow-up care plan. Be sure to discuss any concerns you have about your future physical or emotional health. ASCO offers forms to help keep track of the cancer treatment you received and develop a survivorship care plan when treatment is completed.

This is also a good time to decide who will lead your follow-up care. Some survivors continue to see their oncologist, while others transition back to the care of their family doctor or another health care professional. This decision depends on several factors, including the type and stage of cancer, side effects, health insurance rules, and your personal preferences.

If a doctor who was not directly involved in your cancer care will lead your follow-up care, be sure to share your cancer treatment summary and survivorship care plan forms with him or her and with all future health care providers. Details about your cancer treatment are very valuable to the health care professionals who will care for you throughout your lifetime.

The next section in this guide is Survivorship. It describes how to cope with challenges in everyday life after a cancer diagnosis. Use the menu to choose a different section to read in this guide.

Waldenstrom’s Macroglobulinemia - Survivorship

Approved by the Cancer.Net Editorial Board, 12/2018

ON THIS PAGE: You will read about how to cope with challenges in everyday life after a cancer diagnosis. Use the menu to see other pages.

What is survivorship?

The word “survivorship” means different things to different people. Common definitions include:

  • Having no signs of cancer after finishing treatment.

  • Living with, through, and beyond cancer. According to this definition, cancer survivorship begins at diagnosis and includes people who continue to have treatment over the long term, to either reduce the risk of recurrence or to manage chronic disease.

Survivorship is one of the most complicated parts of having cancer. This is because it is different for everyone.

Survivors may experience a mixture of strong feelings, including joy, concern, relief, guilt, and fear. Some people say they appreciate life more after a cancer diagnosis and have gained a greater acceptance of themselves. Others become very anxious about their health and uncertain about coping with everyday life.

Survivors may feel some stress when their frequent visits to the health care team end after completing treatment. Often, relationships built with the cancer care team provide a sense of security during treatment, and people miss this source of support. This may be especially true when new worries and challenges surface over time, such as any late effects of treatment, emotional challenges including fear of recurrence, sexual health and fertility concerns, and financial and workplace issues.

Every survivor has individual concerns and challenges. With any challenge, a good first step is being able to recognize your fears and talk about them. Effective coping requires:

  • Understanding the challenge you are facing

  • Thinking through solutions

  • Asking for and allowing the support of others

  • Feeling comfortable with the course of action you choose

Many survivors find it helpful to join an in-person support group or an online community of survivors. This allows you to talk with people who have had similar first-hand experiences. Other options for finding support include talking with a friend or member of your health care team, individual counseling, or asking for assistance at the learning resource center of the place where you received treatment.

Changing role of caregivers

Family members and friends may also go through periods of transition. A caregiver plays a very importalint role in supporting a person diagnosed with cancer, providing physical, emotional, and practical care on a daily or as-needed basis. Many caregivers become focused on providing this support, especially if the treatment period lasts for many months or longer.

However, as treatment is completed, the caregiver's role often changes. Eventually, the need for caregiving related to the cancer diagnosis will become much less or come to an end. Caregivers can learn more about adjusting to life after caregiving.

A new perspective on your health

For many people, survivorship serves as a strong motivator to make positive lifestyle changes.

People recovering from Waldenstrom’s macroglobulinemia are encouraged to follow established guidelines for good health, such as not smoking, limiting alcohol, eating well, and managing stress. Regular physical activity can help rebuild your strength and energy level. Your health care team can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about making healthy lifestyle choices.

It is important to have recommended medical checkups and tests (see Follow-up Care) to take care of your health. Cancer rehabilitation may be recommended, and this could mean any of a wide range of services such as physical therapy, career counseling, pain management, nutritional planning, and/or emotional counseling. The goal of rehabilitation is to help people regain control over many aspects of their lives and remain as independent and productive as possible.

Talk with your health care team to develop a survivorship care plan that is best for your needs.

Looking for More Survivorship Resources?

For more information about cancer survivorship, explore these related items. Please note that these links will take you to other sections of Cancer.Net:

  • ASCO Answers Cancer Survivorship Guide: Get this 44-page booklet that helps people transition into life after treatment. It includes blank treatment summary and survivorship care plan forms. The free booklet is available as a PDF, so it is easy to print out.

  • Cancer.Net Patient Education Video: View a short video led by an ASCO expert that provides information about what comes next after finishing treatment.

  • Survivorship Resources: Cancer.Net offers an entire area of this website with resources to help survivors, including for those in different age groups.

The next section offers Questions to Ask the Health Care Team to help start conversations with your cancer care team. Use the menu to choose a different section to read in this guide.

Waldenstrom's Macroglobulinemia - Questions to Ask the Health Care Team

Approved by the Cancer.Net Editorial Board, 12/2018

ON THIS PAGE: You will find some questions to ask your doctor or other members of the health care team, to help you better understand your diagnosis, treatment plan, and overall care. Use the menu to see other pages.

Talking often with the health care team is important to make informed decisions about your health care. These suggested questions are a starting point to help you learn more about your cancer care and treatment. You are also encouraged to ask additional questions that are important to you. You may want to print this list and bring it to your next appointment, or download Cancer.Net’s free mobile app for a digital list and other interactive tools to manage your care.

Questions to ask after getting a diagnosis

  • Can you explain my pathology report, or laboratory test results, to me?

Questions to ask about choosing a treatment and managing side effects

  • What are my treatment options?

  • What clinical trials are available for me? Where are they located, and how do I find out more about them?

  • Can chemotherapy control or cure my cancer?

  • What treatment plan do you recommend? Why?

  • What is the goal of each treatment? Is it to eliminate the cancer, manage my symptoms, or both?

  • Who will be part of my health care team, and what does each member do?

  • Who will be leading my overall treatment and follow-up care?

  • What are the possible short-term and long-term side effects of each treatment?

  • How will this treatment affect my daily life? Will I be able to work, exercise, and perform my usual activities?

  • If I am worried about managing the costs of medical care, who can help?

  • Could this treatment affect my sex life? If so, how and for how long?

  • Could this treatment affect my ability to become pregnant or have children? If so, should I talk with a fertility specialist before cancer treatment begins?

  • How can I keep myself as healthy as possible during and after treatment?

  • Whom should I call with questions or problems?

Questions to ask about planning follow-up care

  • What is the chance that the cancer will come back? Should I watch for specific signs or symptoms?

  • What long-term side effects or late effects are possible based on the treatment I received?

  • What follow-up tests will I need, and how often will I need them?

  • How do I get a treatment summary and survivorship care plan to keep in my personal records?

  • Who will be leading my follow-up care?

  • What survivorship support services are available to me? To my family?

The next section in this guide is Additional Resources. It offers some more resources on this website beyond this guide that may be helpful to you. Use the menu to choose a different section to read in this guide.

Waldenstrom's Macroglobulinemia - Additional Resources

Approved by the Cancer.Net Editorial Board, 12/2018

ON THIS PAGE: You will find some helpful links to other areas of Cancer.Net that provide information about cancer care and treatment. This is the final page of Cancer.Net’s Guide to Waldenstrom’s Macroglobulinemia. To go back and review other pages, use the menu.

Cancer.Net includes many other sections about the medical and emotional aspects of cancer for the person diagnosed and their family members and friends. This website is meant to be a resource for you and your loved ones from the time of diagnosis, through treatment, and beyond.

Here are a few links to help you explore other parts of Cancer.Net:

This is the end of Cancer.Net’s Guide to Waldenstrom’s Macroglobulinemia. Use the menu to select another section to continue reading this guide.