Relapsed/Refractory Waldenstrom Macroglobulinemia

A 42-Year-Old Male With Relapsed/Refractory Waldenström Macroglobulinemia

September 2016

• A 42-year old male presented with blurry vision and nosebleeds.
• Physical examination revealed retinal hemorrhages, adenopathy, and splenomegaly.
• Laboratories revealed a hematocrit of 18% (normal 34.8-43.6%)
  • Platelets of 50,000/mm³(normal 155,000-410,000/mm³)
  • Leukocyte count of 1,500/mm³(normal 3,800-9,200/mm³)
• Serum total protein was high prompting a workup that revealed an IgMλ monoclonal protein and serum IgM level of 12,400 mg/dL.
• CT scans showed bulky adenopathy (> 5 cm)
• A bone marrow biopsy revealed that 80% of the intertrabecular space was involved with lymphoplasmacytic lymphoma.
• Immunohistochemistry demonstrated CD20 expressing bone marrow disease.
• Molecular diagnostic testing for MYD88 and CXCR4 is pending

Treatment

• The patient began several emergent rounds of plasmapheresis, and his vision and energy improved.
• His retinal exam improved and repeat serum IgM level was 3,892 mg/dL.
• The molecular diagnostic studies showed MYD88 L265P and CXCR4 nonsense mutations to be present in the tumor cells.
• The patient received bendamustine alone for two cycles, then rituximab was added to bendamustine for 2 more cycles. He attained a major response.

September 2018

• Two years later, he relapsed with progressive adenopathy (< 5 cm), symptomatic anemia, and his serum IgM rose to 5,459 mg/dL.
• Patient was started on rituximab/ibrutinib combination therapy.