Moffitt Oncology Report Pearls 5/25: Waldenstrom Macroglobulinemia

What is it? IgM monoclonal gammopathy and is categorized as a lymphoplasmacytic lymphoma (not quite like multiple myeloma that has clear plasmytic differentiation!)

 

Clinical features:

  • Cytopenias
  • Lymphadenopathy
  • Cryoglobulinemia
  • Hyperviscosity syndrome
  • End-organ dysfunction from tissue deposition (hepatosplenomegaly)

 

Diagnosis

  • IgM monoclonal gammopathy
  • >= 10% small lymphocytes with plasmacytoid differentiation
  • Immunophenotype (cell surface markers like CD138+ and others)
  • Can be hard to distinguish with other malignancies
    • Waldenstrom vs CLL: CD5 negative if Waldenstrom
    • Waldenstrom vs MM: CD56 negative if Waldenstrom

 

Treatment: the threshold to begin treatment is driven by symptoms and the exact regimen is patient specific based on tolerability considerations (drug side effects) and symptom profile: Bleeding concerns? How sick? Neuropathy?

  • If hyperviscosity syndrome, must start plasmapheresis!!
    • Rituximab can cause an IgM flare in 40-60% of patients and paradoxically worsen hyperviscosity initially!
    • Serum viscosity test >4-6 (normal range 1.4-1.8) is when you can expect hyperviscosity symptoms
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