Composition and significance of splenic Gamna-Gandy bodies in sickle cell anemia

• Published in: Human pathology Vol. 43; no. 7; pp. 1028 - 1036
• Main Authors: Piccin, Andrea, MD, PhD, Rizkalla, Hala, MB Mch, FRCPath, Smith, Owen, MD, McMahon, Corrina, MD, Furlan, Claudio, MD, Murphy, Ciaran, BA Mod, Negri, Giovanni, MD, Mc Dermott, Michael, MB, FRCPath
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01.07.2012; Elsevier; Elsevier Limited
• Subjects: Abdomen; Adolescent; Anemia, Sickle Cell - pathology; Anemia, Sickle Cell - surgery; Anemias. Hemoglobinopathies; Biological and medical sciences; Cellulose acetate; Chemical elements; Child; Child, Preschool; Colleges & universities; Crystals; Diseases of red blood cells; Female; Gamna-Gandy bodies; Hematologic and hematopoietic diseases; Hemorrhage; Humans; Infant; Investigative techniques, diagnostic techniques (general aspects); Male; Medical sciences; Molecular weight; Pathology; Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques; Pneumology; Pulmonary hypertension; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases; Sickle cell anemia; Spectrum analysis; Spleen - pathology; Spleen - surgery; Splenectomy; Splenic Diseases - pathology; Splenic Diseases - surgery; Splenic sequestration; Statistical analysis; Streptococcus infections; Treatment Outcome; Splenectomy; Sickle cell anemia; Splenic sequestration; Pulmonary hypertension; Gamna-Gandy bodies; Spleen; Hemoglobinopathy; Composition; Respiratory disease; Cardiovascular disease; Hemopathy; Genetic disease; Anatomic pathology; Hemolytic anemia; Body
• ISSN: 0046-8177; 1532-8392
• DOI: 10.1016/j.humpath.2011.08.011

Summary 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%; O2 29.7%; P 9.0%; K+ 0.4%; Ca2+ 6.4%; Fe2+ 7.4%), whereas x-ray diffraction studied the structure (CaPO4 ∙ 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.