IgG4 lymphadenopathy


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  • Clinical

    • systemic inflammatory
    • sclerosing lesions in multiple organs
    • classically autoimmune pancreatitis

  • Types

    • I: Castleman disease-like
      • hyperplastic and atrophic follicles
      • interfollicular vascular proliferation
      • eosinophilia
    • II: follicular hyperplasia
      • plasmacytosis
      • scattered eosinophils
    • III: paracortical hyperplasia
      • prominent endothelial venules
      • immunoblasts, plasmablasts, eosinophils
    • IV: progressive transformation of germinal centers
      • expanded mantle zones
      • scattered plasma cells
    • V: inflammatory pseudotumor-like
      • focal fibrous obliteration
      • embedded plasma cells and lymphocytes

  • Stains

    • IgG4 > 100/hpf, IgG4/IgG > 40%
    • polytypic plasma cells

  • Ddx

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