Waldenstrom's macroglobulinemia is a rare disorder that causes the production of abnormal B-lymphocytes, white blood cells. It has some of the same features of both lymphomas and of multiple myeloma. Symptoms are due to accumulation of abnormal lymphocytes in the bone marrow or the abnormal protein in the blood. The former causes anemia, low white blood count or low platelets. These cells also produce too much macroglobulin, a protein that both thickens the blood and coats the platelet cells. The thickened blood may clot in the fingers and toes, causing numbness, or in the brain, causing dizziness and confusion. The covered platelet cells may not work right, resulting in bleeding and easy bruising.
Every year, there are five new cases of macroglobulinemia per 1,000,000 people. The disorder occurs most often in men over the age of 60, but can occur in younger people. The cause of Waldenstrom's Macroglobulinemia is still unknown. There is no known cure.
To diagnose macroglobulinemia, your doctor will do blood tests and, possibly, bone x-rays. If the results are abnormal, you may be referred to a hematologist, a doctor who specializes in diagnosing and treating bone marrow and blood disorders. The hematologist may then test you further by doing a bone marrow biopsy. This consists of removing a small amount of bone marrow and examining it for abnormalities. Special tests on the protein in your blood may also be ordered.
The main goal of treatment is to reduce the amount of abnormal blood/bone marrow cells and protein in the blood. Your treatment plan will be chosen based on your age, health, if you are having any symptoms, and the results of your blood tests and biopsy. Your treatment might include:
In plasmapheresis, the blood is filtered through a machine that removes the abnormal proteins.
Chemotherapy uses medications to regulate the production of B-lymphocytes and their abnormal proteins. Some medications used for this include chlorambucil, cyclophosphamide, fludarabine, vincristine, prednisone, doxorubicin, and 2-Chlorodeoxyadenosine (2-CDA). Your doctor will determine which medication is best for you.
Biological Therapy, or immunotherapy, uses substances to bolster your own immune response to the disorder. Alpha-interferon and rituximab, for example, are medications sometimes used to treat macroglobulinemia.
Bone marrow transplantation for younger patients is sometimes an option although it is experimental.
Managing Waldenstromís Macroglobulinemia
In addition to the treatment recommended by your doctor, there are steps you can take to help reduce or prevent the symptoms of macroglobulinemia:
- Avoid bruising or bumping yourself.
- Use an electric razor when shaving and be cautious when using nail trimmers, knives, etc.
- Wear hard-soled shoes, gloves, and long pants when working outside (i.e. gardening).
- Use a sponge toothbrush if you have problems with gum bleeding. Your doctor or nurse can tell you if you need to use one and where it can be acquired.
- Avoid aspirin or aspirin-like medications (for example, Motrin, ibuprofen, or other anti-inflammatory drugs) unless your doctor has told you otherwise. These medications can affect blood clotting. Also, be sure to inform your doctor of all over-the-counter medications that you take (including vitamins, herbs and dietary supplements).
- Concentrate on having a well-balanced diet during this time. This helps your body to make new red blood cells.
- To conserve energy, sleep and rest between activities.
- Engage in light exercise, such as walking, to stimulate circulation and improve energy levels.
- Perform leg and ankle exercises to prevent clots from forming in the deep veins of the legs. Your doctor or nurse will show you these exercises.
- Maintain an adequate intake of fluids such as water, juices or soda.
- Stop chewing or smoking tobacco. Your doctor or nurse can recommend programs in your community to help you stop.
- Avoid sodium or salt rich foods. These foods may cause fluid retention and make some symptoms worse.
- Breathe deeply and cough periodically. Deep breaths and coughing can help keep your airways open and prevent infection.
- Inform your dentist and all other medical personnel that you have this disorder. There may be increased risk of infection and bleeding during some procedures.
It is important that you be alert for any change or increase in symptoms. If this occurs, it may mean that you need additional or different treatments, and you should contact your doctor right away. The following symptoms will require prompt attention:
- easy bruising
- bleeding for no apparent reason
- unusually heavy or prolonged bleeding
- severe headache or visual changes
- stiff neck
- visual disturbances
- shortness of breath
- pale appearance
- rapid heart rate
- weight loss
- visual changes
- lack of concentration
- confusion in speech
- numbness, tingling, or weakness in arms or legs
- pain or swelling in arms or legs
- shortness of breath
- chest pain
- increased coughing
- coughing up blood
Cardiopulmonary Problem Symptoms
- fullness in the head
- shortness of breath
- weakness and fatigue
- recurrent infections
Follow-Up Care and Prognosis
Waldenstrom's macroglobulinemia requires regular appointments with your doctor. He or she will want to discuss your symptoms and do periodic blood counts to evaluate your response to treatment.
Prognosis depends upon the symptoms you may have, your age, and the treatment you receive. Some patients need minimal supportive care and observation, while other patients may require more intensive treatment.
If you have questions or concerns about your treatment and prognosis, do not hesitate to discuss these with your doctor. It may be helpful to write down your questions before you see the doctor.