View All Pages

Waldenström's Macroglobulinemia - Overview

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will find some basic information about this disease and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Waldenström’s Macroglobulinemia. To see other pages, use the menu on the side of your screen. Think of that menu as a roadmap to this full guide.

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

About lymphoma and the lymph system

Non-Hodgkin lymphoma (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, and T-lymphocytes (also called T-cells) destroy viruses and foreign cells and regulate 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 Waldenström’s macroglobulinemia

Lymphoma begins when cells in the lymphatic system change and grow uncontrollably, which may form a tumor.

In Waldenström’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. Specifically, plasma cells make an antibody called immunoglobulin M (IgM). Another name for Waldenström’s macroglobulinemia is lymphoplasmacytic lymphoma. It is classified as an indolent (low-grade) type of lymphoma. Learn more about the subtypes of NHL.

Because lymphatic tissue is found in so many parts of the body, Waldenström’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, as well as the stomach, intestines, and lungs. Rarely, Waldenström’s macroglobulinemia spreads to the skin or thyroid gland.

Looking for More of an Overview?

If you would like additional introductory information, explore these related items. Please note these links take you to other sections on Cancer.Net:

To continue reading this guide, use the menu on the side of your screen to select another section.  

Waldenström's Macroglobulinemia - Statistics

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will find information about how many people learn they have this type of cancer each year and some general survival information. Remember, survival rates depend on several factors. To see other pages, use the menu on the side of your screen.

Waldenström’s macroglobulinemia is uncommon. Each year, an estimated 1,000 to 1,500 adults in the United States are diagnosed with Waldenström’s macroglobulinemia. The chance of developing Waldenström’s macroglobulinemia increases as people age, and the average age people are diagnosed with Waldenström’s macroglobulinemia is in the late 60s or early 70s.

The five-year survival rate is the percentage of people who survive at least five years after the cancer is detected, excluding those who die from other diseases. The five-year survival rate of people with Waldenström’s macroglobulinemia is about 70%. However, it is important to note that survival rates vary based on a number of individual factors, including the patient’s age, how much the disease has spread at the time of diagnosis, and whether the patient has other medical problems.

Cancer survival statistics should be interpreted with caution. These estimates are based on data from thousands of people with this type of cancer in the United States, but the actual risk for a particular individual may differ. It is not possible to tell a person how long he or she will live with Waldenström’s macroglobulinemia. Learn more about understanding statistics.

Statistics from the American Cancer Society.

To continue reading this guide, use the menu on the side of your screen to select another section.  

Waldenström's Macroglobulinemia - Risk Factors

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will find out more about what factors increase the chance of this type of cancer.  To see other pages, use the menu on the side of your screen.

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. However, 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 Waldenström’s macroglobulinemia:

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

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

Race. White people are more likely to develop Waldenström’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 weakness, tingling, and numbness. However, about 20% of people with MGUS will develop non-Hodgkin lymphoma or multiple myeloma within 20 years.

To continue reading this guide, use the menu on the side of your screen to select another section.  

Waldenström's Macroglobulinemia - Symptoms and Signs

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. To see other pages, use the menu on the side of your screen.

People with Waldenström’s macroglobulinemia may experience the following symptoms or signs. Sometimes, people with Waldenström’s macroglobulinemia do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom or sign on this list, please talk with your doctor.   

  • Fatigue
  • Unexplained weight loss
  • Enlarged lymph nodes or spleen
  • Weakness, numbness, or other nervous system problems
  • Abdominal swelling and diarrhea
  • Weakness and shortness of breath
  • Infections
  • Raised, fleshy-colored lesions on the skin
  • Changes in the color of the finger tips when exposed to cold
  • Changes in vision

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

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. Most patients report that either their nightclothes or the sheets on the bed are actually wet.
  • Itchiness

Symptoms of hyperviscosity (thick blood)

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
  • Confusion
  • Dizziness
  • Loss of coordination
  • Headaches
  • Nosebleeds and bleeding gums
  • Fatigue

Your doctor will ask you questions about the symptoms you are experiencing to help find out the cause of the problem, called a diagnosis. This may include how long you’ve been experiencing the symptom(s) and how often.

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

The next section helps explain what tests and scans may be needed to learn more about the cause of the symptoms. Use the menu on the side of your screen to select Diagnosis, or you can select another section, to continue reading this guide.

Waldenström's Macroglobulinemia - Diagnosis

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will find a list of the common tests, procedures, and scans that doctors can use to find out what’s wrong and identify the cause of the problem. To see other pages, use the menu on the side of your screen.

Doctors use many tests to diagnose cancer and find out if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most types of cancer, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the cancer has spread. This list describes options for diagnosing this type of cancer, and not all tests listed will be used for every person. Your doctor may consider these factors when choosing a diagnostic test:

  • Age and medical condition
  • Type of cancer suspected
  • Signs and symptoms
  • Previous test results

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

Blood tests. Blood tests, such as CBC-D (complete blood count with differential count) and an examination of the blood with a microscope, can help your doctor diagnose Waldenström’s macroglobulinemia. There is also an increase in IgM that can be detected by protein electrophoresis (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. The sample removed during the biopsy is analyzed by a pathologist (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 Waldenström’s macroglobulinemia is either a bone marrow biopsy (see below) 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 (see below) to guide the doctor, or from the stomach or intestine during an endoscopy. An endoscopy is a test that allows the doctor to see inside the body using a thin, lighted, flexible tube called an endoscope.

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 Waldenström’s macroglobulinemia, and it can help determine if the cancer has spread.

A bone marrow aspiration and biopsy are similar and often done at the same time. Bone marrow has both a solid and a liquid part. A bone marrow biopsy is the removal of a small amount of solid tissue using a needle. An aspiration removes a sample of fluid with a needle. The sample(s) are then analyzed by a pathologist. A common site for a bone marrow biopsy and aspiration is the pelvic bone, which is located in the lower back by the hip. The skin in that area is numbed with a local anesthetic beforehand (medication to block the awareness of pain), and other types of anesthesia may be used. 

Molecular testing. Your doctor may recommend running laboratory tests on a tissue sample to identify specific genes, proteins, and other factors unique to the tumor. Results of these tests will help decide whether your treatment options include a type of treatment called targeted therapy (see the Treatment Options section).

CT or CAT scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities, tumors, enlarged lymph nodes, or a swollen spleen. A CT scan can also be used to measure the size of a tumor, lymph node, or spleen. Sometimes, a contrast medium (a special dye) is injected into a patient’s vein or given orally (by mouth) to provide better detail. 

Magnetic resonance imaging (MRI) scan. An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium (a special dye) may be injected into a patient’s vein or given orally to create a clearer picture.

Positron emission tomography (PET) scan. A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive substance is injected into a patient’s body. This substance is absorbed mainly by organs and tissues that have higher metabolic activity (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.

After these 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 cancer; this is called staging.

The next section helps explain the different stages for this type of cancer. Use the menu on the side of your screen to select Stages, or you can select another section, to continue reading this guide.  

Waldenström's Macroglobulinemia - Stages

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will learn about how doctors usually describe a cancer’s growth or spread, called the stage, and how this differs for Waldenström’s macroglobulinemia. To see other pages, use the menu on the side of your screen.

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 determine 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 (chance of recovery). There are different stage descriptions for different types of cancer.

There is no standard system for staging Waldenström’s macroglobulinemia. Instead, doctors may look at several factors to determine a prognosis, such as the severity of a patient’s anemia (low level of red blood cells), the amount of IgM in the blood, and the amount of a protein in the blood called beta-2-microglobulin. People with normal hemoglobin (the protein that carries oxygen in a red blood cell) levels and low beta-2-microglobulin levels typically have a better prognosis.

Information about the cancer’s stage will help the doctor recommend a treatment plan.  The next section helps explain the treatment options for this type of cancer. Use the menu on the side of your screen to select Treatment Options, or you can select another section, to continue reading this guide.  

Waldenström's Macroglobulinemia - Treatment Options

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will learn about the different ways doctors use to treat people with this type of cancer. To see other pages, use the menu on the side of your screen.

This section outlines treatments that are the standard of care (the best proven treatments available) for this specific type of cancer. When making treatment plan decisions, patients are also encouraged to consider clinical trials as an option. A clinical trial is a research study to test a new treatment to evaluate whether it is safe, effective, and possibly better than the standard treatment. Your doctor can help you review all treatment options. For more information, 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.

Descriptions of the most common treatment options for Waldenström’s macroglobulinemia are listed below. 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 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. Learn more about making treatment decisions.

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.

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.

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.

Learn more about chemotherapy and preparing for treatment. 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.

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. Learn more about targeted treatments.

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.

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. Learn more about bone marrow and stem cell transplantation.

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. Learn more about palliative care.   

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. Learn more about coping with the fear of recurrence

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. Learn more about dealing with cancer recurrence.

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. This discussion may include clinical trials.

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.

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. 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 bereavement.

The next section helps explain clinical trials, which are research studies. Use the menu on the side of your screen to select About Clinical Trials, or you can select another section, to continue reading this guide. 

Waldenström's Macroglobulinemia - About Clinical Trials

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

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. To see other pages, use the menu on the side of your screen.

Doctors and scientists are always looking for better ways to treat patients with Waldenström’s macroglobulinemia. To make scientific advances, doctors create research studies involving volunteers, called clinical trials.

Many clinical trials are focused on new treatments, evaluating whether a new treatment is safe, effective, and possibly better than the current (standard) treatment. These types of studies evaluate new drugs, different combinations of existing treatments, new approaches to chemotherapy or surgery, and new methods of treatment. Patients who participate in clinical trials are often among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.

There are also clinical trials that study new ways to ease symptoms and side effects during treatment and manage the late effects that may occur after treatment. Talk with your doctor about clinical trials regarding side effects. In addition, there are ongoing studies about ways to prevent the disease.

Patients decide to participate in clinical trials for many reasons. For some patients, 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 patients volunteer for clinical trials because they know that these studies are the only way to make progress in treating Waldenström’s macroglobulinemia. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with Waldenström’s macroglobulinemia.

Sometimes people have concerns that, by participating in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” The use of placebos in cancer clinical trials is rare. 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.

To join a clinical trial, patients must participate in a process known as informed consent. During informed consent, the doctor should list all of the patient’s options so that the person understands the standard treatments, and how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different than the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment.

For specific topics being studied for Waldenström’s macroglobulinemia, learn more in the Latest Research section.

Patients 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 patients 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 the patient chooses to leave the clinical trial before it ends. 

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.

The next section helps explain the areas of research going on today about this type of cancer. Use the menu on the side of your screen to select Latest Research, or you can select another section, to continue reading this guide. 

Waldenstrom's Macroglobulinemia - Latest Research

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

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. To see other pages, use the menu on the side of your screen.

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

New treatments.  These therapies are being explored in clinical trials:

  • Sildenafil (Viagra, Revatio) stops several proteins that certain types of cancer need to grow and spread, and laboratory tests have shown that it can keep Waldenström’s macroglobulinemia from worsening.
  • Vaccines have been studied in clinical trials as a treatment option for Waldenström’s macroglobulinemia.

Targeted therapy. As explained in the Treatment Options section, targeted therapy can block tumor growth in different ways.

  • Alemtuzumab (Campath) is being studied for its ability to locate and destroy tumor cells or to deliver other substances to the tumor cells than can destroy them without harming normal cells.
  • Bortezomib (Velcade) is a type of targeted therapy approved for multiple myeloma called a proteasome inhibitor, targeting specific enzymes called proteasomes that digest proteins in the cells. Bortezomib and other proteasome inhibitors, such as carfilzomib (Kyprolis), and oprozomib are being studied for the treatment of Waldenström’s macroglobulinemia.
  • Other drugs that target how B-cells develop are also being evaluated for patients with Waldenström’s macroglobulinemia. Ibrutinib inhibits the Bruton tyrosine kinase enzyme and has shown to be effective in early clinical trials. Idelalisib inhibits PI3 kinase, which is involved in B-cell growth, and is being evaluated for a variety of B-cell cancers, including Waldenström’s macroglobulinemia. The drugs everolimus (Afinitor) and temsirolimus (Torisel), as well as drugs called histone deacetylase (HDAC) inhibitors, such as panobinostat, are also being looked at as treatments for Waldemstrom’s macroglobulinemia.

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

  • Monoclonal antibodies, such as rituximab (see Treatment Options), 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 Waldenström’s macroglobulinemia, as is another combination of drugs called DTPACE (dexamethasone [multiple brand names], thalidomide [Synovir, Thalomid], cisplatin [Platinol], doxorubicin [Adriamycin], cyclophosphamide, and etoposide [Toposar, VePesid]) plus rituximab.
  • Bortezomib, dexamethasone, and rituximab are being studied in combination to treat Waldenström’s macroglobulinemia.

Supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current Waldenström’s macroglobulinemia treatments in order to improve patients’ comfort and quality of life.

Looking for More about the Latest Research?

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

The next section addresses how to cope with the symptoms of the disease or the side effects of its treatment. Use the menu on the side of your screen to select Coping with Side Effects, or you can select another section, to continue reading this guide.

Waldenstrom's Macroglobulinemia - Coping with Side Effects

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will find out more about steps to take to help cope with physical, social, and emotional side effects. This page includes several links outside of this guide to other sections of this website. To see other pages, use the menu on the side of your screen.    

Fear of treatment side effects is common after a diagnosis of cancer, but it may help to know that preventing and controlling side effects is a major focus of your health care team. This is called palliative or supportive care, and it is an important part of the overall treatment plan, regardless of the stage of disease.

Common side effects from each treatment option for Waldenström’s macroglobulinemia are described in detail within the Treatment Options section. Learn more about the most common side effects of cancer and different treatments, along with ways to prevent or control them. Side effects depend on a variety of factors, including the cancer’s stage, the length and dosage of treatment(s), and your overall health.

Before treatment begins, talk with your doctor about possible side effects of each type of treatment you will be receiving. Ask which side effects are most likely to happen, when they are likely to occur, and what can be done to prevent or relieve them. And, ask about the level of caregiving you may need during treatment and recovery, as family members and friends often play an important role in the care of a person with Waldenström’s macroglobulinemia. Learn more about caregiving.

In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Patients and their families are encouraged to share their feelings with a member of their health care team who can help with coping strategies. Learn more about the importance of addressing such needs, including concerns about managing the cost of your cancer care

During and after treatment, be sure to tell the health care team about the side effects you experience, even if you feel they are not serious. Sometimes, side effects can last beyond the treatment period, called a long-term side effect. A side effect that occurs months or years after treatment is called a late effect. Treatment of both types of effects is an important part of survivorship care. Learn more by reading the After Treatment section or talking with your doctor.

The next section helps explain medical tests and check-ups needed after finishing cancer treatment. Use the menu on the side of your screen to select After Treatment, or you can select another section, to continue reading this guide.  

Waldenström's Macroglobulinemia - After Treatment

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

ON THIS PAGE: You will read about your medical care after cancer treatment is finished and why this follow-up care is important. To see other pages, use the menu on the side of your screen.

After treatment for Waldenström’s macroglobulinemia ends, talk with your doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your recovery for the coming months and years. Normally, follow-up visits are most frequent in the first three years after treatment, but patients are encouraged to receive lifelong follow-up care. ASCO offers cancer treatment summary forms to help keep track of the cancer treatment you received and develop a survivorship care plan once treatment is completed.

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

  • Lung damage from certain types of chemotherapy.
  • Heart damage in the form of a weakened heart muscle may occur in people who have received a higher dose of doxorubicin.
  • Secondary cancers (other cancers that develop because of the original cancer treatments) are more common in people who received chemotherapy.

People recovering from Waldenström’s macroglobulinemia are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, not smoking, eating a balanced diet, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. Moderate physical activity can help rebuild your strength and energy level. Your doctor can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about the next steps to take in survivorship, including making positive lifestyle changes.

The next section offers a list of questions you may want to ask. Use the menu on the side of your screen to select Questions to Ask the Doctor, or you can select another section, to continue reading this guide.

Waldenström's Macroglobulinemia - Questions to Ask the Doctor

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

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

Talking often with the doctor 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 an e-list and other interactive tools to manage your care.

  • What is the stage of my disease? What does this mean?
  • Can you explain my pathology report (laboratory test results) to me?
  • What are my treatment options?
  • What clinical trials are open to me?
  • Can chemotherapy control my cancer?
  • What is the goal of each treatment? Is it to eliminate the cancer, manage my symptoms, or both?
  • What treatment plan do you recommend? Why?
  • What are the possible short-term and long-term side effects of each treatment?
  • Who will be part of my health care team, and what does each member do?
  • Who will be coordinating my overall treatment and follow-up care?
  • How will this treatment affect my daily life? Will I be able to work, exercise, and perform my usual activities?
  • 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?
  • If I’m worried about managing the costs related to my medical care, who can help me with these concerns?
  • How can I keep myself as healthy as possible during and after treatment?
  • What follow-up tests will I need, and how often will I need them?
  • What support services are available to me? To my family?
  • Whom should I call for questions or problems?

The next section offers some more resources that may be helpful to you. Use the menu on the side of your screen to select Additional Resources, or you can select another section, to continue reading this guide.

Waldenström's Macroglobulinemia - Additional Resources

This section has been reviewed and approved by the Cancer.Net Editorial Board, 09/2013

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 Waldenström’s Macroglobulinemia. To go back and review other pages, use the menu on the side of your screen.

Cancer.Net includes many other sections about the medical and emotional aspects of cancer, both 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 sections that may get you started in exploring the rest of Cancer.Net:


This is the end of Cancer.Net’s Guide to
Waldenström’s Macroglobulinemia. Use the menu on the side of your screen to select another section to continue reading this guide.