Waldenstrom Macroglobulinemia

Introduction

• B cell lymphoma with monoclonal IgM production
• type of non-Hodgkin lymphoma

Presentation

• Presents in older individuals
• Symptoms 
  • constitutional symptoms
    • fatigue and headache
  • generalized lymphadenopathy
  • petechial bleeding
    • IgM disrupts platelet aggregation
  • anemia
  • Raynaud phenomenon
  • sequelae of blood hyperviscosity and cold agglutinins
    • retinal hemorrhage
    • stroke
• decreased renal function

Evaluation

• Labs
  • ↑ ESR, LDH, alkaline phosphatase, and uric acid
• Bone marrow biopsy
  • abnormal plasma cells 
    • contains intranuclear aggregations of IgM (Dutcher bodies)
• Serum protein electrophoresis 
  • M spike = IgM 
    • unlike multiple myeloma which is IgA/G
• Urine
  • Bence Jones proteins will be present
    • Ig light chains
  • urine dipstick will be negative for protein as it detects only albumin
• Imaging
• no lytic bone lesions

Treatment

• Symptomatic only
  • rituximab
• Plasmapheresis when hyperviscosity is present