Waldenstrom Macroglobulinemia

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Plasma Cell Dyscrasias

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Summary

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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