How is hyperviscosity syndrome treated in patients with Waldenström macroglobulinemia?

Updated: Feb 01, 2021
  • Author: Karen Seiter, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Hyperviscosity syndrome manifestations should be treated promptly, and emergent care is paramount. The treatment of choice for symptoms related to hyperviscosity is urgent plasmapheresis. [33] The principle behind management is that 80% of all IgM is confined to the intravascular space. Most often, half of the volume or more should be removed to significantly lower the serum viscosity.

Viscosity should be measured before and after plasmapheresis. Approximately 2-4U of plasma must be removed every 1-2 weeks because the effects produced are not permanent and plasma is replaced with albumin and saline.

Chemotherapy should be considered soon after stabilization to reduce the production of the paraprotein by the malignant lymphocytes.

Macroglobulinemia can cause complications similar to peripheral neuropathy; cryoglobulinemia or amyloidosis can occur in the absence of high IgM concentrations and manifestations of the lymphomatous process. These symptoms largely result from certain physicochemical properties of the monoclonal IgM protein and can be treated by repeated plasmapheresis followed by systemic therapy. However, evidence supporting plasma exchange for the treatment of peripheral neuropathy associated with IgM paraprotein is weak (grade of recommendation C).

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