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