Department of Haematology and BMT Unit, San Maurizio Regional Hospital, Bolzano 39100, Italy.
Hum Pathol. 2012 Jul;43(7):1028-36. doi: 10.1016/j.humpath.2011.08.011. Epub 2011 Nov 12.
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.
镰状细胞贫血患儿可能会发生急性脾脏扣押。脾切除术是为了减少进一步的发作而进行的。对脾脏的组织学研究揭示了存在肉芽肿样结节，称为 Gamna-Gandy 体，具有无定形内含物；然而，其意义尚不清楚。回顾了 2001 年至 2007 年间在都柏林的 Our Lady's Children's Hospital 接受脾切除术治疗的连续镰状细胞贫血患儿的病历记录和组织学样本。确定了 17 名患者。通过扫描电子显微镜和 X 射线荧光光谱研究了 Gamna-Gandy 体。在 7 名（41%）患者中发现了 Gamna-Gandy 体，并且总是可见无定形内含物。患者年龄与 Gamna-Gandy 体显著相关（P =.002）。扫描电子显微镜分析表明 Gamna-Gandy 体具有结晶性质和化学成分（C 47.1%；O(2) 29.7%；P 9.0%；K(+) 0.4%；Ca(2+) 6.4%；Fe(2+) 7.4%），而 X 射线衍射研究了结构（CaPO(4) ∙ FeOH）。观察到从红髓到白髓的晶体形成梯度。我们的研究表明 Gamna-Gandy 体含有晶体，其形成与年龄有关。我们还证明了晶体结构和化学成分，以及 Gamna-Gandy 体与脾切除前或脾切除后胸部危机之间的关系。
