Waldenstrom macroglobulinemia (mak-roe-glob-u-lih-NEE-me-uh) is a rare type of cancer that begins in the white blood cells.

If you have Waldenstrom macroglobulinemia, your bone marrow produces too many abnormal white blood cells that crowd out healthy blood cells. The abnormal white blood cells produce a protein that accumulates in the blood, impairs circulation and causes complications.

Waldenstrom macroglobulinemia is considered a type of non-Hodgkin's lymphoma. It's sometimes called lymphoplasmacytic lymphoma.

Waldenstrom macroglobulinemia is slow growing and may not cause signs and symptoms for many years.

When they do occur, signs and symptoms may include:

• Easy bruising
• Bleeding from the nose or the gums
• Fatigue
• Weight loss
• Numbness in your hands or feet
• Fever
• Headache
• Shortness of breath
• Changes in vision
• Confusion

When to see a doctor

Make an appointment with your doctor if you have any persistent signs and symptoms that worry you.

It's not clear what causes Waldenstrom macroglobulinemia.

Doctors know that the disease begins with one abnormal white blood cell that develops errors (mutations) in its genetic code. The errors tell the cell to continue multiplying rapidly.

Because cancer cells don't mature and then die as normal cells do, they accumulate, eventually overwhelming production of healthy cells. In the bone marrow — the soft, blood-producing tissue that fills in the center of most of your bones — Waldenstrom macroglobulinemia cells crowd out healthy blood cells.

Waldenstrom macroglobulinemia cells continue trying to produce antibodies, as healthy white blood cells do, but instead they produce abnormal proteins that the body can't use. The protein immunoglobulin M (IgM) accumulates in the blood, impairs circulation and causes complications.

Factors that may increase your risk of Waldenstrom macroglobulinemia include:

• Being older. Waldenstrom macroglobulinemia can occur at any age, but it's most often diagnosed in adults 65 and older.
• Being male. Males are more likely to be diagnosed with Waldenstrom macroglobulinemia.
• Being white. White people are more likely to develop the disease, compared with people of other races.
• Having a family history of lymphoma. If you have a relative who has been diagnosed with Waldenstrom macroglobulinemia or another type of B-cell lymphoma, you may have an increased risk.

Tests and procedures used to diagnose Waldenstrom macroglobulinemia include:

• Blood tests. Blood tests may reveal low numbers of healthy blood cells. Also, blood tests are used to detect the IgM proteins produced by the cancer cells.

  Blood tests may also measure your organ function, which can tell your doctor whether the IgM proteins are affecting your organs, such as your kidneys and your liver.

• Collecting a sample of bone marrow for testing. During a bone marrow biopsy, your doctor uses a needle to extract some of your bone marrow from your hipbone. The sample is examined to look for cancer cells. If any are detected, advanced laboratory analysis can help your doctor understand the cancer cells' characteristics, including their genetic mutations.
• Imaging tests. Imaging tests can help your doctor determine whether cancer has spread to other areas of your body. Imaging tests may include computerized tomography (CT) scans or positron emission tomography (PET) scans.

Treatment options for Waldenstrom macroglobulinemia may include:

• Observation. If IgM proteins are found in your blood, but you don't have any signs or symptoms, you may choose to wait before beginning treatment. Your doctor may recommend blood tests every few months to monitor your condition. You may go years without needing further treatment.
• Plasma exchange. If you experience signs and symptoms related to having too many IgM proteins in your blood, your doctor may recommend plasma exchange (plasmapheresis) to remove the proteins and replace them with healthy blood plasma.

• Chemotherapy. Chemotherapy is a drug treatment that kills quickly growing cells, such as the abnormal blood cells produced by Waldenstrom macroglobulinemia.

  Chemotherapy may be used alone or combined with other drug treatments as an initial treatment for people who experience signs and symptoms of Waldenstrom macroglobulinemia. High-dose chemotherapy may also be used to suppress your bone marrow production in preparation for a bone marrow transplant.

• Targeted therapy. Targeted therapy drugs kill cancer cells by focusing on the specific abnormalities present in the cancer cells that allow them to survive. Targeted therapy drugs may be used alone or combined with other medications, such as chemotherapy or biological therapy, as an initial treatment for Waldenstrom macroglobulinemia or in cases where the cancer returns despite treatment.
• Biological therapy. Biological therapy drugs use your immune system to kill cancer cells. Biological therapy drugs can be used alone or in combination with other medications as an initial treatment or as a treatment for recurrent Waldenstrom macroglobulinemia.
• Bone marrow transplant. In certain highly selected situations, a bone marrow transplant, also known as a stem cell transplant, may be used to treat Waldenstrom macroglobulinemia. During this procedure, high doses of chemotherapy are used to wipe out your diseased bone marrow. Healthy blood stem cells are infused into your body where they can rebuild healthy bone marrow.
• Clinical trials. Clinical trials give you a chance to try the latest in Waldenstrom macroglobulinemia treatment.

If you have any signs or symptoms that worry you, make an appointment with your doctor.

If you're diagnosed with Waldenstrom macroglobulinemia, you'll likely be referred to a doctor who specializes in treating blood and bone marrow disorders (hematologist) or a doctor who specializes in treating cancer (oncologist).

Because appointments can be brief and because there's often a lot of ground to cover, it's a good idea to be well-prepared. Here's some information to help you get ready and know what to expect from your doctor.

What you can do

• Note symptoms you're experiencing. If you have had signs and symptoms of illness or are just not feeling well, write down those details before your appointment. Your doctor will also want to know when you first noticed these symptoms and whether they've changed over time.
• Make a list of your medications. Include any prescription or over-the-counter medications you're taking, as well as all vitamins, supplements and herbal remedies.
• Take a family member or friend along. Sometimes it can be difficult to remember all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.
• Write down questions to ask your doctor.

Questions to ask your doctor at your initial appointment include:

• What may be causing my symptoms or condition?
• Are there any other possible causes?
• What kinds of tests do I need?
• What do you recommend for next steps in determining my diagnosis and treatment?
• Are there any restrictions that I need to follow in the meantime?

Questions to consider if your doctor refers you to a specialist include:

• Do I have Waldenstrom macroglobulinemia?
• What are the goals of treatment in my case?
• What treatment do you recommend?
• Is it necessary to begin treatment right away?
• I have these other health problems. How can I best treat them together?
• What are the possible side effects of treatment?
• If the first treatment isn't successful, what will we try next?
• Am I a candidate for bone marrow transplantation?
• What is the outlook for my condition?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask questions during your appointment at any time that you don't understand something.

What to expect from your doctor

Your doctor is likely to ask you a number of questions. Thinking about your answers ahead of time can help you make the most of your appointment. Your doctor may ask:

• What are your symptoms, if any?
• When did you first begin experiencing symptoms?
• How have your symptoms changed over time?
• Have you been diagnosed or treated for any other medical conditions?
• What medications are you taking?