Histopathological features of chronic granulomatous disease (CGD) in childhood
Article first published online: 21 OCT 2005
Volume 47, Issue 5, pages 508–516, November 2005
How to Cite
Levine, S., Smith, V. V., Malone, M. and Sebire, N. J. (2005), Histopathological features of chronic granulomatous disease (CGD) in childhood. Histopathology, 47: 508–516. doi: 10.1111/j.1365-2559.2005.02258.x
- Issue published online: 21 OCT 2005
- Article first published online: 21 OCT 2005
- Date of submission 10 June 2005 Accepted for publication 14 July 2005
- chronic granulomatous disease;
Aims : To describe the spectrum of histopathological features encountered in children with chronic granulomatous disease (CGD) at a specialist centre.
Methods and results : The histopathological findings of 88 surgical pathology requests from a range of organ systems including upper and lower gastrointestinal tract biopsy series, liver, bladder, bone, lung, skin, soft tissue, bone marrow and lymph node biopsy specimens, in 32 patients aged 4 months to 18 years (median 7 years) with CGD were reviewed. In most tissues the features were those of active chronic inflammation, with or without abscess or granuloma formation, often associated with fungal infection. In some tissues, more characteristic findings were identified, including the presence of pigmented macrophages, especially in hepatic sinusoids and colonic mucosa, where active chronic eosinophil predominant colitis was also observed.
Conclusions : Chronic granulomatous disease may present to histopathologists in a wide range of tissue specimens most often demonstrating features of active chronic inflammation with or without granuloma formation. The presence of numerous pigmented macrophages in association with such inflammation should raise suspicion of the diagnosis. In addition, diffuse granulomatous inflammation of the lung and hepatic abscess formation should be regarded as suggestive of the diagnosis.