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• W2416361415 abstract "An 11-year-old boy was admitted to the hospital with complaints of fatigue and anorexia lasting for 6 years. No reliable history about his natal and postnatal period was available. On physical examination failure to thrive was detected (height: third percentile, weight: third to 10th percentile). His skin and conjunctivas were pale and his abdomen was distended. The spleen and liver were 2 cm and 12 cm palpable, respectively. On laboratory investigations, leukopenia (3.0 × 109/L), thrombocytopenia (94.0 × 109/L) and iron deficiency anemia were detected. His serum protein, albumin, aspartate aminotransferase and alanine aminotransferase levels were 7.9 g/dL, 4.6 g/dL, 29 U/L and 20 U/L, respectively. The International normalized ratio was 1.1. Abdominal ultrasonography showed splenomegaly, hepatomegaly and portal venous collateral formation (cavernous transformation) in porta hepatis. There were focal hyperechoic spots within the spleen (Fig. 1). The portal vein was thrombosed on color Doppler imaging. Computed tomography scans revealed collateral vasculature in porta hepatis and hepatosplenomegaly without parenchymal heterogeneity in the spleen and liver. Magnetic resonance imaging of T1 and T2-weighted sequences revealed small hypointense spots within the spleen and portal venous collaterals (Fig. 2a). Intravenous contrast (gadolinium-diethylenetriaminepentaacetate) enhanced images demonstrated these spots more clearly than nonenhanced images (Fig. 2b). On upper gastrointestinal endoscopy, grade 3 esophageal varices were detected; endoscopic band ligation was performed.FIG. 1: Abdominal ultrasonography shows splenomegaly and focal hyperechoic spots within the spleen (arrows).FIG. 2: a, An axial fat-suppressed T1-weighted image reveals small hypointense spots (white arrow) within the spleen with thrombosed portal vein (PV) and vascular collaterals (black arrows). b, An intravenous contrast (gadolinium-DTPA) enhanced fat-suppressed T1-weighted image demonstrates these spots more clearly than nonenhanced images (arrow).What is the most likely explanation for these nodules? a. Splenic angiosarcoma. b. Gamna-Gandy bodies. c. Langerhans cell histiocytosis. d. Splenic metastasis. ANSWER/DISCUSSION Gamna-Gandy bodies (GGB) are foci of iron deposits in the splenic parenchyma resulting from intraparenchymal hemorrhage, commonly seen in patients with liver cirrhosis and portal hypertension (1). In portal hypertension, although the prolonged transit time of the blood causes pancytopenia, bleeding into the red pulpa with deposition of siderin results in lesions called GGB (1). Classically, these lesions are of low signal intensity or signal void in all T1- and T2-weighted magnetic resonance images and result in susceptibility artifacts. Because magnetic resonance imaging is very sensitive to paramagnetic agents such as deoxyhemoglobin, methemoglobin and hemosiderin, magnetic resonance imaging seems to be the superior imaging method for detection of these lesions owing to the properties of GGB (1). In two different series with portal hypertension, GGB were detected in 9.0% to 12.5% of the cases (1,2). These nodules were most apparent on images obtained with the GRASS (gradient-recalled acquisition in the steady state) technique, which is known to be more sensitive to heterogeneity of the magnetic susceptibility, usually caused by hemosiderin deposition (2). We found that gradient-echo sequences and fat-suppressed images were more sensitive, and the image quality for siderotic nodules within the spleen were superior on IV contrast-enhanced images than standard spin-echo sequences. On ultrasonography, GGB are seen as bright hyperechoic small nodules. One study reported that of 34 patients with GGB, only 24 were detected with ultrasonography (3). It was also demonstrated that unenhanced computerized tomography could also detect GGB in only a very limited number of patients; this entity should be included in the differential diagnosis of calcified foci of the spleen (4). Splenic angiosarcomas with siderotic nodules were reported; thus in the differential diagnosis it should be kept in mind (5). Studies agree that the degree of splenomegaly is not related to the frequency of GGB (1). Consequently, images of GGB were introduced to the literature with advanced imaging techniques and neither ultrasonography nor computed tomography are reliable techniques for the diagnosis of GGB. Magnetic resonance imaging may be the imaging method of choice to detect GGB because of its sensitivity to iron-containing structures. Most pediatricians are unaware of their occurrence because of the very few reports in literature and the lack of knowledge in classic pediatric textbooks. Moreover, no pediatric case with GGB has previously been reported. We report this case to confirm the occurrence of GGB in the pediatric age group and to warn pediatricians to avoid further investigations to enlighten the etiology of these small nodules when coincidentally encountered." @default.
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• W2416361415 title "Clinical Quiz" @default.
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• W2416361415 doi "https://doi.org/10.1097/01.mpg.0000154011.02796.4d" @default.
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