The following case study focuses on a 65-year-old woman with a history of Waldenström
macroglobulinemia that has gone untreated who presents with a two-week history
of headaches, blurred vision, and bruising. Test your knowledge by reading the
background information below and making the proper selection.
What is the most appropriate initial therapy?
a. R-CHOP x 6-8 cycles
d. Rituximab weekly x 4 doses
This patient has hyperviscosity syndrome secondary to Waldenström
macroglobulinemia that requires urgent plasmapheresis. Waldenström is a
lymphoplasmacytic lymphoma associated with an elevated circulating monoclonal
IgM. Of the immunoglobulin classes, IgM is most associated with
hyperviscosity because of its pentamer structure. Symptoms of
hyperviscosity include headaches, vision changes, mental status changes,
seizures, and bleeding. Congestive heart failure and volume overload can
also be seen.
been used successfully in Waldenström, however, initial therapy should be
plasmapheresis. Other options for frontline combinations include
fludarabine combinations, rituximab + cyclophosphamide + dexamethasone, and
bortezomib combinations. Steroids are unlikely to produce a quick response
and would not provide a durable remission, so they are not a good choice for
initial therapy. Rituximab as a single agent can be considered in low-risk
patients; however, a flare of IgM can be seen with this agent, and so it should
not be used in patients who have any symptoms suggestive of hyperviscosity.
- Gertz MA. Waldenström macroglobulinemia: 2011 update on
diagnosis, risk stratification, and management. Am J Hematol. 2011;86:411-416.
- Treon SP. How I treat Waldenström macroglobulinemia. Blood.
- Ghobrial IM, Fonseca R, Greipp PR, et al. Initial
immunoglobulin M ‘flare’ after rituximab therapy in patients diagnosed with
Waldenstrom macroglobulinemia: An Eastern Cooperative Oncology Group Study. Cancer.
submitted by Jennifer Woyach, MD, The Ohio State University.
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