Patients with IgM MGUS (Monoclonal Gammopathy of Undetermined Significance) and SWM (Smoldering Waldenstrom's Macroglobulinemia) do not need to be treated.
Although patients with WM need to be monitored regularly, they should not be treated until symptoms develop.
Indications for therapy include constitutional symptoms (weakness, fatigue, night sweats, fever or weight loss) and symptomatic conditions such as cryoglobulinemia, cold agglutinin disease, peripheral neuropathy, and amyloidosis. Progressive, symptomatic enlargement of the lymph nodes, liver, or spleen is also an indication for treatment. A hemoglobin level less than 10 g/dL or a platelet count less than 100,000 due to bone marrow infiltration also justifies therapy.
Hyperviscosity syndrome requires urgent treatment. Treatment consists of plasmapheresis, 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 (usually with albumin and sodium chloride solutins), and return of the remaining blood components to the body. This results in a reduction of the serum IgM levels. However, plasmapheresis is of only temporary benefit and the patient needs to repeat the process in a few weeks. Consequently, patients with hyperviscosity syndrome are started on systemic treatment.
Patients exhibiting masses of WM cells outside the bone marrow (extramedullary masses) may need to initiate treatment based on their disease burden, as well as the location, size, and rate of growth of the masses. Urgency of treatment will also depend on these factors.
For more detailed information on treatment therapies please see WM Treatment Primer.