Lymphoma is the most common blood cancer. The two main forms of lymphoma are Hodgkin lymphoma and non-Hodgkin lymphoma (NHL). Lymphoma occurs when cells of the immune system called lymphocytes, a type of white blood cell, grow and multiply uncontrollably. Cancerous lymphocytes can travel to many parts of the body, including the lymph nodes, spleen, bone marrow, blood, or other organs, and form a mass called a tumor. The body has two main types of lymphocytes that can develop into lymphomas: B-lymphocytes (B-cells) and T-lymphocytes (T-cells).
Waldenström macroglobulinemia (also known as lymphoplasmacytic lymphoma or immunocytoma) is a rare, indolent (slow-growing) B-cell lymphoma that occurs in less than two percent of patients with NHL. There are about 1,500 new cases of Waldenström each year. The disease usually affects older adults and is primarily found in the bone marrow, although lymph nodes and the spleen may be involved.
Patients with Waldenström have a high level of a protein called immunoglobulin M (IgM) in their blood. These high levels of IgM can cause a thickening of the blood (hyperviscosity), resulting in symptoms such as nosebleeds, headaches, dizziness, and blurring or loss of vision. Other symptoms may include tiredness, night sweats, headaches, pain or numbness in the extremities, and increased size of the liver, spleen, and lymph nodes. While some patients experience these symptoms, others do not.
To diagnose Waldenström, blood and urine tests are usually performed as well as a bone marrow biopsy. During the biopsy, a needle is inserted into a bone (usually the pelvic bone) and a small sample of bone marrow is extracted for examination.
For patients without symptoms, doctors may decide not to treat the disease right away, an approach referred to as "watch and wait" or "watchful waiting." Watch and wait can last for many years for some patients. The type and severity of an individual's symptoms, such as thickness of the patient's blood, age, and overall health play a role in the type of treatment selected. The choice of treatment is based on individual patient needs, as well as considerations for short-term and long-term side effects.
Some patients receive a procedure called plasmapheresis to reverse or prevent the symptoms associated with the thickening of the blood. This procedure involves removing the patient’s blood, passing it through a machine that removes the part of the blood containing the IgM antibody, and returning the blood to the patient. Doctors often combine plasmapheresis with other treatments, such as chemotherapy.
There are many drugs that can be used to manage this disease, including single-agents such as rituximab (Rituxan), chlorambucil (Leukeran), cladribine (Leustatin), fludarabine (Fludara), bortezomib (Velcade), bendamustine (Treanda) alone and in various combinations.
For many Waldenström patients, there is a delayed response to the treatment they receive. The best response to treatment is sometimes achieved several months after the treatment ends.
Waldenström is an incurable disease, although many patients have a long-term response to treatment. For patients who relapse (disease returns after treatment) or become refractory (disease does not respond to initial treatment), secondary therapies may be successful in providing additional remissions. The previous therapies discussed can be used or re-used depending on length of remission, patient's age, stem cell transplant eligibility, or previous toxicities encountered. Additional therapies to treat refractory/relapsed Waldenström, include:
On January 29, 2015 the U. S. Food and Drug Administration approved ibrutinib (Imbruvica Capsules) for the treatment of patients with Waldenström macroglobulinemia (WM). The approval was based on demonstration of durable responses in a single arm, multi-center clinical trial enrolling 63 patients with previously treated WM. For further information, please visit the U.S. Food and Drug Administration.
Several new drugs and drug combinations are being studied in clinical trials for Waldenström macroglobulinemia (some for refractory/relapsed disease), including: • Carfilzomib and • LBH-589
It is critical to remember that today's scientific research is continuously evolving. Treatment options may change as new treatments are discovered and current treatments are improved. Therefore, it is important that patients check with LRF or with their physician for any treatment updates that may have recently emerged.
Clinical trials are crucial in identifying effective drugs and determining optimal doses for lymphoma patients. Patients interested in participating in a clinical trial should talk to their physician or contact LRF's Helpline for an individualized clinical trial search by calling (800) 500-9976 or emailing firstname.lastname@example.org.
Patients in remission should have regular visits with a physician who is familiar with their medical history as well as the treatments they have received. Patients will likely have some medical tests (such as blood tests and computed axial tomography [CAT] scans) at various times during remission so that the physician can evaluate whether additional treatment is needed.
Some treatments can cause long-term effects or late effects, which can vary based on duration and frequency of treatments, age, gender, and the overall health of each patient at the time of treatment. The doctor will check for these effects during follow-up care. Visits may become less frequent the longer the disease remains in remission.
Survivors and their caregivers are encouraged to keep copies of all medical records and test results as well as information on the types, amounts, and duration of all treatments received. This documentation will be important for keeping track of any effects resulting from treatment or potential disease recurrences. For further information, please review our fact sheet on survivorship issues.
LRF offers a wide range of resources that address treatment options, the latest research advances, and ways to cope with all aspects of lymphoma. LRF also provides many educational activities, from in-person meetings to teleconferences and webcasts. For more information about any of these resources, visit the website at www.lymphoma.org or contact the Helpline at (800) 500-9976 or email@example.com.
View or order the following publications from our booklets/factsheets webpage: