Waldenstrom Macroglobulinemia

Summary

Coming soon...

Key Points

• Waldenstrom Macroglobulinemia
  • Cause
    • Plasma cell cancer that overproduces IgM
      • Named “macroglobulinemia” because IgM is the largest Ig (pentameric)
  • Presentation
    • No bone lesions or hypercalcemia
      • Excludes multiple myeloma
    • Peripheral neuropathy
    • Hyperviscosity syndrome
      • Headache
      • Blurry vision
      • Raynaud phenomenon
      • Retinal hemorrhages
    • Can lead to thrombosis (e.g. stroke)
    • Anemia
      • Excess plasma cell growth in bone marrow can crowd out normal hematopoiesis
      • Less common than in multiple myeloma
  • Diagnosis
    • M-spike in serum
      • Detected via serum protein electrophoresis (SPEP)
      • Represents overproduction of monoclonal Ig fragment
    • Bence-Jones protein in urine
      • Ig light chain accumulation is known as Bence-Jones protein
      • Detected via 24-hour urine collection and protein electrophoresis
      • Note: rapid urine dipstick is negative (only detects albumin)
    • >10% plasma cells on bone marrow biopsy
      • with IgM-containing vacuoles (lymphoplasmacytic lymphoma)
  • Treatment
    • Still area of active research
    • Rituximab
    • Bortezomib (protease inhibitor) may have utility
    • Plasmapheresis if hyperviscosity syndrome is present