Patients should be treated when they have symptoms and not on the basis of blood test results alone. This applies not only to consideration of initial treatment but also to treatment following relapse, which is sometimes called salvage therapy. Many therapies have toxic side effects, and treating patients who don’t yet have symptoms may potentially have an adverse effect on quality of life and health that is worse than the disease itself at that point.
The consensus opinion of WM experts concludes that the following symptoms or conditions are considered appropriate reasons for therapy:
- Hyperviscosity syndrome (excessive thickness of the blood due to IgM) – requires urgent treatment with plasmapheresis (PP), a process which involves removal of blood from the body, separation of the plasma portion containing the IgM from the blood, replacement of the plasma volume, and return of the remaining blood components to the body. PP temporarily reduces the serum IgM levels; however, it does not affect the underlying disease and the patient must repeat the process in a few weeks. For that reason, patients with hyperviscosity syndrome are usually started on systemic treatment to reduce their disease burden.
- Anemia due to infiltration of the bone marrow with WM cells – the most frequent condition requiring treatment. Generally speaking, a hemoglobin level less than 10 g/dL is an indication to begin therapy.
- A platelet count less than <100,000 (called thrombocytopenia) due to bone marrow infiltration.
- Constitutional symptoms – weakness, fatigue, night sweats, fever, or weight loss.
- Conditions such as cryoglobulinemia, cold agglutinin disease, peripheral neuropathy, and amyloidosis. A brief description of these conditions is listed in our Signs and Symptoms section.
- Progressive, symptomatic enlargement of the lymph nodes, liver, or spleen.
- Kidney disease (nephropathy) related to WM.
- Masses of WM cells outside the bone marrow (extramedullary masses) – treatment may be initiated based on the location, size, and rate of growth of the masses.