American Society of Hematology

Case Study: 65-Year-Old with History of Waldenstrom Macroglobulinemia

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
b. Plasmapheresis
c. Steroids
d. Rituximab weekly x 4 doses

Answer

b. Plasmapheresis

Explanation

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.

R-CHOP has 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.

Further Reading

  1. Gertz MA. Waldenström macroglobulinemia: 2011 update on diagnosis, risk stratification, and management. Am J Hematol. 2011;86:411-416.
  2. Treon SP. How I treat Waldenström macroglobulinemia. Blood. 2009;114:2375-2385.
  3. 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. 2004;101:2593-2598.

Case study submitted by Jennifer Woyach, MD, The Ohio State University.

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