People with WM may have no symptoms for years after diagnosis. There does not seem to be a correlation between the level of monoclonal IgM and the degree of symptoms. Patients with similar laboratory test results may have markedly different types and degrees of symptoms. When symptoms do appear, they may be totally different from one person to another.
Before your diagnosis, WM is normally preceded by IgM MGUS or smoldering WM (SWM).
IgM MGUS is characterized by an IgM monoclonal protein less than 3.0 g/dL; bone marrow containing less than 10% lymphoplasmacytic cells; absence of symptomatic anemia, enlarged lymph nodes, liver or spleen; absence of hyperviscosity (increased thickness of the blood); and no constitutional symptoms (weakness, fatigue, weight loss, night sweats or fever). Only 1.5 % of patients with IgM MGUS will progress to WM or a related disorder each year.
SWM is characterized by the presence of a serum IgM monoclonal protein equal to or greater than 3.0 g/dL and/or 10% or more lymphoplasmacytic cells in the bone marrow. These patients have no symptoms–no significant anemia, hyperviscosity, constitutional symptoms or symptomatic enlargement of the lymph nodes, liver or spleen. Patients with SWM progress to WM or a related disorder at a rate of 11% per year for the first 5 years and then the risk decreases.
Clinical Features and Complications
Patients with WM may present with features including those listed below.
There are certain recurring problems that physicians find in WM patients:
- Tiredness, usually the result of anemia
- Night sweats
- Headaches and dizziness
- Various visual problems
- Pain, numbness, or tingling in the extremities
- Abnormal bleeding from the nose and gums
- Enlarged lymph nodes, spleen, liver
Almost one-fourth of patients with WM will present with neurologic symptoms and findings. Peripheral neuropathy is characterized by numbness and tingling of the feet or hands. While the feet are more commonly involved than the hands, the symptoms involve all extremities and are usually symmetric. Weakness of the legs and arms may develop.The neuropathy is slowly progressive.
This refers to the involvement of the retina, fundus or eye grounds. Your physician or an ophthalmologist may see enlargement of the veins in your retina (back of the eye). In addition, hemorrhages may also be seen. Segmentation ("sausaging") of the vessels may be observed. These retinal changes are the best indication of hyperviscosity (thickening of the blood).
Anemia is a common feature of WM and oftentimes is the cause of symptoms leading the patient to see a physician. The patient may complain of weakness, fatigue, lightheadedness, palpitation of the heart or shortness of breath.
Hyperviscosity means increased thickness of the blood. This is due to large amounts of the IgM protein circulating in the blood. Hyperviscosity syndrome may produce headache, blurred vision, dizziness, vertigo, ataxia (loss of coordination) double vision, seizures and reduced consciousness. The serum viscosity level can be measured in the laboratory, but it does not correlate well with symptoms of hyperviscosity. Examination of the retina by your physician or ophthalmologist is a much better measure of hyperviscosity.
Cold Agglutinin disease
About 10 percent of WM patients have an acquired hemolytic anemia called cold agglutinin disease. In cold agglutinin disease, monoclonal IgM causes the destruction of red blood cells when a patient is in an environment where temperatures are low. Raynaud’s phenomenon, exemplified by signs of poor circulation in the fingers, is an indicator of cold agglutinin disease. Red cell circulation in blood vessels in the skin of the nose, ears, fingers and toes may be affected by the cold air temperatures.
Ten to 20 percent of patients with WM also develop cryoglobulinemia, a condition that is made worse by exposure to cold temperatures. Cryoglobulinemia is manifested by Raynaud’s phenomenon, kidney problems, and purpura (purplish or red-brown discoloration of the skin).
This is a group of diseases caused by the buildup of an abnormal protein, called amyloid, in various tissues and organs of the body, such as the heart, kidneys, nervous system, or gastrointestinal tract. The amyloid protein forms fibers that may injure these body parts or interfere with their normal functioning. The protein may be deposited in a localized area or throughout the body and may be hereditary or caused by inflammation or certain cancers. Although amyloidosis is rare in WM, it occurs when the light chain portions of the abnormal IgM protein remain undissolved in the bloodstream and are deposited in one or more organs.
You can learn more at the website of the Amyloidosis Foundation.
Other Clinical Features
Infections are more common than in the normal population but not as common as in multiple myeloma. Occasionally the lymphoplasmacytic cells of WM will involve the lung and produce masses or pleural effusion (fluid in the chest). Diarrhea is uncommon. Involvement of the kidneys is rare. Occasionally patients will have urticaria (hives) or rarely the lymphoplasmacytic cells may infiltrate the skin. Patients with WM may have increased risk of bleeding because the IgM protein may interfere with platelet function and normal clotting factors. Involvement of bone is rare in comparison to that in multiple myeloma.
A small number of patients may exhibit masses of WM cells in various parts of the body. These masses have been reported in the extremities, the spine, the breast, the eye socket, and the palate. In the case of spinal tumors, additional symptoms could include numbness, back pain, tingling, and weakness in the lower extremities as well as difficulties in bowel or bladder function.