Waldenstrom’s Macroglobulinemia, also called lympho-plasmacytic lymphoma or immunocytoma, is a rare, slow-growing b-cell lymphoma that occurs in less than 2 percent of people with NHL. There are about 1,500 new cases of Waldenstrom’s each year. The disease usually affects older adults and is found primarily in the bone marrow, although lymph nodes and the spleen may be involved.
Waldenstrom’s is characterized by a high level of a protein called immunoglobulin in the blood, which can cause a thickening of the blood and result in 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.
Waldenstrom’s is diagnosed with blood and urine tests as well as with a bone marrow biopsy. For patients without symptoms, patients often take a watch and wait approach.
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 or combinations of drugs that can be used to manage this disease, including: single-agents such as chlorambucil (Leukeran); cCladribine or fludarabine; rituximab (Rituxan) or a combination such as R CHOP; R cyclophosphamide dexamethasone; R fludarabine; R bendamustine (Treanda); or R bortezomib (Velcade).
The choice of treatment is based on individual patient needs, as well as considerations for short and long-term side effects. In patients with high IgM levels, rituximab should be used with caution due to a potential flare phenomenon leading to abrupt increases in IgM levels and aggravation of serum viscosity. In patients, with an IgM level of 5000 mg/dL or higher or who have hyper viscosity symptoms (such as headaches, blurry vision, nosebleeds), plasmapheresis may be performed before rituximab is used, and IgM levels should then be closely followed. In younger, stem cell transplant eligible patients the use of chlorambucil and nucleoside analogue agents such as fludarabine or cladribine should be avoided due to possible toxicity to stem cells. An increased risk of secondary cancers has also been reported with chlorambucil and nucleoside analogues. An increased risk of neuropathy is associated with vincristine and bortezomib based therapy.