Original contributionComposition and significance of splenic Gamna-Gandy bodies in sickle cell anemia
Introduction
Acute splenic sequestration occurs in children as young as 5 weeks, but it is seen most often in children between the age of 3 months and 5 years and represents a life-threatening situation in sickle cell anemia (SCA) [1], [2]. It is caused by acute pooling of blood cells in the spleen, trapping erythrocytes, and is defined by hemoglobin decrease of at least 2 g/dL, associated with elevated reticulocyte count and an acutely enlarged spleen [3]. The classic clinical signs are splenomegaly, abdominal pain, abdominal tenderness, pallor, and irritability. Treatment is based on top-up transfusion or red blood cell exchange. Transfusion delay could lead to hypovolemic shock and death [4]. Since the introduction of efficacious vaccinations against capsulated bacteria (eg, Haemophilus influenzae, Streptococcus pneumoniae, etc), splenectomy (SP) has become an accepted procedure in the management of SCA patients undergoing recurrent splenic crisis.
Histologic studies of SCA spleens have shown the presence of granuloma-like nodules, known as Gamna-Gandy bodies (GGBs) (Fig. 1). The true significance of GGBs and their inclusion is still unknown; the early hypothesis formulated by C. Gamna that GGBs originate by periarterial hemorrhage followed by fibrosis and impregnation of iron pigments is to date the most credible [5]. Several pathologic reports from the early 20th century have attempted to provide a very accurate description of this phenomenon; however, these articles are difficult to access because the PubMed database contains medical citations only from 1950 onward. In addition, the current literature is limited to a few radiologic case reports correlated with simplistic histopathologic descriptions of the phenomenon. In this study, all articles on GGB published after 1905 in several languages were reviewed.
We present a series of pediatric cases of splenic GGB in SCA. The morphological slide review was correlated with a detailed scanning electron microscopy (SEM) with x-ray fluorescence analysis (XRF) in energy dispersive spectroscopy (EDS) mode of the histopathologic samples using high and low magnification [6]. In addition, XRF by SEM allowed the identification of the chemical constituents of GGB crystals and their distribution within splenic architecture.
Section snippets
Patients
The medical notes of all consecutive SCA children undergoing open SP at the National Tertiary Sickle Centre, at Our Lady's Children's Hospital, Dublin, between 2001 and 2007 were reviewed. All patients had been diagnosed with SCA by high-performance liquid chromatography and cellulose acetate hemoglobin electrophoresis at pH 8.6. The number and type of crises (bone, chest, abdominal, etc) pre- and post-SP were recorded. Blood samples obtained 4 weeks before SP in the absence of transfusion were
Patients
Seventeen patients were identified. All were of African ethnicity: 14 (82%) Nigerian, 2 (11%) Congolese, and 1 (5%) Angolese. Eleven patients were male and 6 were female (male/female = 1.8). Sixteen patients were diagnosed with HbSS, and 1 with, HbSC. The median age at SP was 4 years and 3 months (range, 10 months to 15 years). A consistent trend of raised WBC, N, and platelets post-SP was found. Painful bone crises were increased after SP, but patients treated with hydroxyurea before SP did
Discussion
The first GGB description is a case report of a patient with biliary cirrhosis. GGBs were found in the spleen, but no interpretation on their nature was given [8]. In 1910, Billtroff [9] reported GGB-like lesions in the lung vessels of a man who died of endocarditis. For the first time, the siderotic nature of GGB was suggested; however, the etiology was erroneously believed to be mycotic. In 1921, a report by Italian pathologist Carlo Gamna [5] finally shed light on the nature of GGB. The
Acknowledgments
We are grateful to Prof. Antonio Marigo, Dr Carla Marega and Dr Valerio Causin (Department of Chemical Sciences, University of Padua) for their contribution in the microspectrographic analysis. We are also in debt to the Biblioteca Pinali Sezione Antica from Padua for providing access to antique articles and books.
Contributions: A. P. designed the study, wrote the manuscript, treated patients, collected data, reviewed literature, and performed statistical analysis; H. R. reviewed histologic
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