title: Composition and significance of splenic Gamna-Gandy bodies in sickle cell anemia.
abstract: Children with sickle cell anemia may undergo acute splenic sequestration. Splenectomy is performed in an attempt to reduce further events. Histologic studies of spleens have revealed the presence of granuloma-like nodules, known as Gamna-Gandy bodies with amorphous inclusions; however, their significance is unknown. The medical case records and histologic samples of consecutive children with sickle cell anemia treated with splenectomy between 2001 and 2007 at Our Lady's Children's Hospital, Dublin, were reviewed. Seventeen patients were identified. Gamna-Gandy bodies were studied by scanning electron microscopy and x-ray fluorescence spectroscopy. Gamna-Gandy bodies were identified in 7 (41%) patients, and amorphous inclusions were always seen. Patient age correlated significantly with Gamna-Gandy bodies (P = .002). Scanning electron microscopic analysis demonstrated the crystalline nature of Gamna-Gandy bodies and the chemical composition (C 47.1%; O(2) 29.7%; P 9.0%; K(+) 0.4%; Ca(2+) 6.4%; Fe(2+) 7.4%), whereas x-ray diffraction studied the structure (CaPO(4) ∙ FeOH). A crystal-formation gradient was observed, increasing from the red pulp to the white pulp. Our study shows that Gamna-Gandy bodies contain crystals and that their formation is age dependent. We also demonstrated the crystal structure and chemical composition and the relationship between Gamna-Gandy bodies and chest crises presplenectomy or postsplenectomy.
authors.affiliation: Department of Haematology and BMT Unit, San Maurizio Regional Hospital, Bolzano 39100, Italy. andrea.piccin@asbz.it
authors.forename: Andrea
authors.initials: A
authors.lastname: Piccin
authors.forename: Hala
authors.initials: H
authors.lastname: Rizkalla
authors.forename: Owen
authors.initials: O
authors.lastname: Smith
authors.forename: Corrina
authors.initials: C
authors.lastname: McMahon
authors.forename: Claudio
authors.initials: C
authors.lastname: Furlan
authors.forename: Ciaran
authors.initials: C
authors.lastname: Murphy
authors.forename: Giovanni
authors.initials: G
authors.lastname: Negri
authors.forename: Michael
authors.initials: M
authors.lastname: Mc Dermott
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000473:pathology* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D013154:Spleen / Q000473:pathology* / Q000601:surgery; D013156:Splenectomy; D013158:Splenic Diseases / Q000473:pathology* / Q000601:surgery; D016896:Treatment Outcome