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

Piccin, Andrea; Rizkalla, Hala; Smith, Owen; McMahon, Corrina; Furlan, Claudia; Murphy, C.; Negri, Giovanni; Dermott, Michael Mc

doi:10.1016/j.humpath.2011.08.011

Cited by 26 publications

(37 citation statements)

References 15 publications

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“…Piccin et al (2) observed that patients with SCA and GGBs had low platelet counts, which was consistent with the results of the present study. This indicated that the presence of splenic siderotic nodules corresponded with the occurrence of thrombocytopenia in patients with liver cirrhosis.…”

Section: Discussionsupporting

confidence: 93%

“…A histological study on splenic GGBs in patients with SCA demonstrated that GGBs were only detected by microscopy in patients >4 years of age, and that patients younger than this did not develop GGBs. Furthermore, hemosiderin deposits were observed in all patients, whereas microscopy revealed the presence of GGBs in 58% of cases (2). Hemosiderin deposition, old hemorrhages and GGBs may coexist and correspond with the different stages of hemolysis diseases, as well as liver cirrhosis, with hemorrhage and hemosiderin occurring in the early stages and GGBs appearing later.…”

Section: Discussionmentioning

confidence: 93%

“…Splenic GGBs have been described to appear in a number of conditions, including chronic hemolysis, portal hypertension, sickle cell anemia (SCA), leukemia and lymphoma. It has been suggested that GGBs derive from red cell breakdown, and are a chronic, late event occurring following hemorrhage and red cell destruction (2). A histological study on splenic GGBs in patients with SCA demonstrated that GGBs were only detected by microscopy in patients >4 years of age, and that patients younger than this did not develop GGBs.…”

Section: Discussionmentioning

confidence: 99%

“…GGBs have been revealed to measure a few millimeters in size and be composed of hemosiderin, calcium and fibrous tissue, with a crystal structure of CaPO 4 .FeOH. The iron inclusions favor the deposition of calcium salts (1,2). …”

Section: Introductionmentioning

confidence: 99%

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Splenic siderotic nodules in patients with liver cirrhosis

Ouyang¹,

Gong²,

Zha³

et al. 2013

Experimental and Therapeutic Medicine

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The aim of this study was to investigate the interrelation between splenic siderotic nodules, hypersplenism and liver function in patients with liver cirrhosis. The splenic enhanced susceptibility-weighted angiography (ESWAN) and conventional magnetic resonance images of 33 patients with liver cirrhosis were retrospectively studied and the ESWAN images were graded. The distribution and prevalence of the image grades for patients with and without hypersplenism were evaluated. In addition, the splenic volume and the distribution of Child-Pugh and albumin scores were compared between patients with and without siderotic nodules, and the correlation between splenic volume and the ESWAN image grades were evaluated in the patients with siderotic nodules. The ESWAN images revealed splenic siderotic nodules in 24 patients. The distribution and prevalence of the ESWAN image grades were demonstrated to be significantly different (P<0.001) between patients with and without hypersplenism. Furthermore, significant differences were observed between patients with and without siderotic nodules with regard to splenic volume and the distribution of Child-Pugh and serum albumin scores (P<0.001). No significant correlation was demonstrated between splenic volume and the ESWAN image grades (P>0.05). In conclusion, a higher prevalence of splenic siderotic nodules (72.7%) was observed using the ESWAN sequence, in comparison with results from previous studies, obtained using the T1-spoiled gradient echo sequence. The presence of splenic siderotic nodules was consistent with the occurrence of hypersplenism and was interrelated with reserved liver function.

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Section: Discussionsupporting

confidence: 93%

Section: Discussionmentioning

confidence: 93%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Splenic siderotic nodules in patients with liver cirrhosis

Ouyang¹,

Gong²,

Zha³

et al. 2013

Experimental and Therapeutic Medicine

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“…Furthermore, previous SCA studies have already documented that microparticle generation occurs and may participate in the haemolytic process, with release of free haemoglobin and iron [3]. Piccin et al [4] demonstrated the presence of iron-based compounds in splenic inclusions in children with SCA undergoing recurrent crises, also known as Gamna-Gandy bodies. Interestingly, the presence of Gamna-Gandy bodies has also been reported in patients with haemochromatosis [5].…”

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confidence: 99%

Endothelial Microparticles and Endothelial Damage: ‘The Tip and the Iceberg'

Piccin¹

2014

Acta Haematol

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Comparative histological analysis of spleens in pediatric patients with hemolytic anemias: Insights into the pathophysiological mechanisms of spleen destruction in sickle cell anemia

Brousse,

El Hoss,

Isnard

et al. 2024

American J Hematol

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While sickle cell anemia (SCA) and hereditary spherocytosis (HS) share common features of increased spleen erythrophagocytosis due to increased red blood cell (RBC) turnover, SCA is specifically characterized by susceptibility to infections. In this study, histological lesions in the spleens of pediatric patients with SCA were analyzed, in close correlation with past clinical history and comparatively to HS, healthy and transfused β‐thalassemia patients (TDT). An evaluation of red pulp elementary lesions (red pulp fibrosis, iron deposition, number of Gandy–Gamna, and RBC trapping) combined into a severity score was established, as well as B‐cell follicles analysis. Quantification on digitalized slides of iron deposition, RBC trapping, and red pulp fibrosis was additionally performed. Spleens from 22 children with SCA, eight with HS, eight with TDT, and three healthy controls (HC) were analyzed. Median age at splenectomy was not different between SCA and HS patients, 6.05 years (range: 4.5–16.0) versus 4.75 (range: 2.2–9.5). Marked heterogeneity was found in SCA spleens in contrast to other conditions. Contrary to previous reports, B‐cell follicles were generally preserved in SCA. While RBC trapping was significantly increased in both SCA and HS (compared to TDT and HC), quantitative fibrosis and overall red pulp severity score were significantly increased in SCA spleens compared to other conditions. Moreover, there was an inverse correlation between quantitative fibrosis and number of B‐cell follicles, linking these two compartments as well as spleen fibrosis to infectious susceptibility in SCA, potentially through impaired red pulp macrophage scavenging and B‐cell subpopulations defects.

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

Cited by 26 publications

References 15 publications

Splenic siderotic nodules in patients with liver cirrhosis

Splenic siderotic nodules in patients with liver cirrhosis

Endothelial Microparticles and Endothelial Damage: ‘The Tip and the Iceberg'

Comparative histological analysis of spleens in pediatric patients with hemolytic anemias: Insights into the pathophysiological mechanisms of spleen destruction in sickle cell anemia

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