Waldenstrom Hyperglobulinemic Purpura

  • Gammopathy, less aggressive variant
 
Mechanism
  • Circulating immune complexes that contain IgG or IgA rheumatoid factor
    • May have underlying autoimmune connective tissue disorder (most likely Sjogren)
 
Clinical presentation
  • Episodic showers of petechiae occurring on all parts of the body (lower extremities more often)
  • Dorsum of feet intensely involved
  • Aggravated by prolonged standing or walking
 
Labs
  • Serum electrophoresis shows a broad based peak (IgG mostly but IgA may be seen, IgM low or normal)
  • Up to 80% have (+) SSA/Ro Abs