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GLILD

Monday 29 October 2007

Group of nodular infllammatory lesions observed in common variable immunodeficiency (CVID). Approximately 10% of patients with common variable immune deficiency (CVID) have a systemic granulomatous disease with associated interstitial lung disease.

Synopsis

- pulmonary lymphoid hyperplasia

  • BALT hyperplasia (follicular bronchiolitis)
    • bronchiolocentric lymphoid follicles with germinal centers
  • lymphoid hyperplasia
    • lymphoid follicles with germinal centers with a widespread distribution, often along lymphatic pathways and interlobular septa
  • lymphocytic interstitial pneumonia (nodular LIP)
    • nodular lymphocytic interstitial pneumonia (nodular LIP)
    • LIP is characterized by diffuse interstitial inflammatory infiltrates consisting of mature lymphocytes, plasma cells, and histiocytes.

- non-necrotizing granulomatous lung disease

  • occasional clusters of epithelioid histiocytes
  • loose clusters of epithelioid histiocytes, multinucleated giant cells, and lymphocytes

- organizing pneumonia
- B-cell mucosa-associated tissue lymphoma (pulmonary marginal zone lymphoma)

References

- Wheat WH, Cool CD, Morimoto Y, Rai PR, Kirkpatrick CH, Lindenbaum BA, Bates CA, Ellison MC, Serls AE, Brown KK, Routes JM. Possible role of human herpesvirus 8 in the lymphoproliferative disorders in common variable immunodeficiency. J Exp Med. 2005 Aug 15;202(4):479-84. PMID: #16103407#

- Bates CA, Ellison MC, Lynch DA, Cool CD, Brown KK, Routes JM. Granulomatous-lymphocytic lung disease shortens survival in common variable immunodeficiency. J Allergy Clin Immunol. 2004 Aug;114(2):415-21. PMID: #15316526#

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