What is Waldenstrom macroglobulinemia (lymphoplasmacytic lymphoma)?
Waldenstrom macroglobulinemia (WM) is a cancer of blood cells. It is classified as a lymphoma because it arises from mature B-lymphocytes (aka, “B cells”). These cells grow uncontrollably primarily in the bone marrow, but also in the lymph nodes, liver and spleen in some cases. These cells also overproduce an IgM antibody.
Waldenstrom’s macroglobulinemia, a form of lymphoma, is most often found in white males age 65 or over. It is considered to be an indolent lymphoma, spreading slowly. If detected early, Waldenstrom can be controlled easily.
Waldenstrom’s macroglobulinemia cancer cells are similar to those in multiple myeloma and non-Hodgkin’s lymphoma. In Waldenstrom’s, the body produces too much of a protein called immunoglobulin M (IgM). When this protein builds up, it can cause the blood to become thick, making it difficult to move through the blood vessels.
How Waldenstrom macroglobulinemia forms
The manifestations of WM are due to both growth of the cancerous B cells and the overproduction of the antibody. The growth of the cancerous B cells in the bone marrow leads to low blood counts. The growth of the cancerous B cells can also lead to enlarged liver, spleen and lymph nodes throughout the body.
Overproduction of the IgM antibody may cause the blood to become thick, a condition called hyperviscosity. This condition makes it difficult for blood to flow throughout certain parts of the body. The antibody may also attack certain tissues in the body and cause inflammation or injury.
Under the microscope, WM cells also have features of plasma cells, which are the most mature type of B cells. For this reason, WM cells are considered to be “lymphoplasmacytic”.
Waldenstrom macroglobulinemia is a rare condition diagnosed in less than 1,500 people in the US annually.
Symptoms of Waldenstrom’s disease
Symptoms of Waldenstrom’s may include some or all of the following:
- Decrease in vision
- Weight loss without trying
- Numbness in extremities
- Tiredness
- Bluish skin
- Swollen spleen, glands
- Fatigue and weakness
- Low red blood cell count (anemia), which may cause weakness and dizziness
- Low white blood cells (leukopenia), which may cause frequent infections
- Low platelet count (thrombocytopenia), which may cause frequent bleeding
Waldenstrom macroglobulinemia risk factors
A risk factor is a characteristic that can make someone more likely to develop a disease. Some risk factors like age and sex cannot be modified or lowered. The cause of WM is unknown, but here are factors that may increase your risk:
- Age: The risk for developing Waldenstrom’s grows with age; most people diagnosed are over the age of 60.
- Sex: More men than women develop Waldenstrom’s.
- Race: Waldenstrom’s is more common in Caucasians.
- Having IgM MGUS
Having a condition called IgM monoclonal gammopathy of undetermined significance (MGUS) may also increase your risk for developing Waldenstrom macroglobulinemia.
In people with IgM MGUS, their bone marrow has abnormal lymphoplasmacytic cells and the IgM antibody is detectable in the blood, but they do not have symptoms or other abnormalities. The majority of people with MGUS will live with the condition without developing symptomatic WM.
How is Waldenstrom macroglobulinemia diagnosed?
The following tests and diagnostic procedures may be used to diagnose Waldenstrom’s macroglobulinemia:
- A physical exam and full medical history evaluation including urine test
- A complete blood count (CBC) that will examine the number of red blood cells and platelets, and the number and type of white blood cells. The CBC will also examine other portions of the blood.
- Blood chemistry studies
- Bone marrow biopsy with chromosomal testing
- Cytogenetic analysis
- Imaging studies such as PET scans, CT scans, x-rays and MRI studies
Waldenstrom macroglobulinemia diagnosis at Penn Medicine
Waldestrom macroglobulinemia treatment
There is no cure for Waldenstrom macroglobulinemia, but people can live with Waldenstrom’s for many years with treatment because Waldenstrom’s is slow-growing, or indolent.
Waldenstrom macroglobulinemia treatment at Penn Medicine
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