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Liver iron content determination by magnetic resonance imaging

World Journal of Gastroenterology 2010年第13期16卷 1587-1597页

作者： Konstantinos Tziomalos vassilios perifanis 1st Propedeutic Department of Internal Medicine Medical School Aristotle University of Thessaloniki AHEPA University Hospital Thalassemia Unit Hippokration Hospital

Accurate evaluation of iron overload is necessary to establish the diagnosis of hemochromatosis and guide chelation treatment in transfusion-dependent anemia. The liver is the primary site for iron storage in patients with hemochromatosis or transfusion-dependent anemia, therefore, liver iron concentration (LIC) accurately re? ects total body iron stores. In the past 20 years, magnetic resonance imaging (MRI) has emerged as a promising method for measuring LIC in a variety of diseases. We review the potential role of MRI in LIC determination in the most important disorders that are characterized by iron overload, that is, thalassemia major, other hemoglobinopathies, acquired anemia, and hemochromatosis. Most studies have been performed in thalassemia major and MRI is currently a widely accepted method for guiding chelation treatment in these patients. However, the lack of correlation between liver and cardiac iron stores suggests that both organs should be evaluated with MRI, since cardiac disease is the leading cause of death in this population. It is also unclear which MRI method is the most accurate since there are no large studies that have directly compared the different available techniques. The role of MRI in the era of genetic diagnosis of hemochromatosis is also debated, whereas data on the accuracy of the method in other hematological and liver diseases are rather limited. However, MRI is a fast, non-invasive and relatively accurate diagnostic tool for assessing LIC, and its use is expected to increase as the role of iron in the pathogenesis of liver disease becomes clearer.

关键词： Thalassemia major Iron overload Magnetic resonance imaging Liver Hemochromatosis Desferrioxamine Deferiprone Deferasirox Thalassemia inter media Myelodysplastic syndromes

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A case of successful management with splenectomy of intractable ascites due to congenital dyserythropoietic anemia type II-induced cirrhosis

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World Journal of Gastroenterology 2006年第5期12卷 818-821页

作者： Themistoklis Vassiliadis Vassilia Garipidou vassilios perifanis Konstantinos Tziomalos OIga Giouleme Kalliopi Patsiaoura Michalis Avramidis Nikolaos Nikolaidis Sofia Vakalopoulou Ioannis Tsitouridis Antonios Antoniadis Panagiotis Semertzidis Anna Kioumi Evangelos Premetis Nikolaos Eugenidis 2~(nd) Propedeutic Department of Internal Medicine Aristotle University of ThessalonikiHippokration General HospitalThessalonikiGreece Department of Pathology Hippokration General HospitalThessalonikiGreece Department of Radiology Papageorgiou General HospitalThessalonikiGreece Organ Transplant Unit Aristotle University of ThessalonikiHippokration General HospitalThessalonikiGreece Department of Hematology Papageorgiou General Hospital Thessaloniki Greece Hematology Laboratory Aghia Sophia Children's HospitalAthensGreece

The congenital dyserythropoietic anemias comprise a group of rare hereditary disorders of erythropoiesis, characterized by ineffective erythropoiesis as the predominant mechanism of anemia and by characteristic morphological aberrations of the majority of erythroblasts in the bone marrow. Congenital dyserythropoietic anemia type II is the most frequent type. All types of congenital dyserythropoietic anemias distinctly share a high incidence of iron loading. Iron accumulation occurs even in untransfused patients and can result in heart failure and liver cirrhosis. We have reported about a patient who presented with liver cirrhosis and intractable ascites caused by congenital dyserythropoietic anemia type II. Her clinical course was further complicated by the development of autoimmune hemolytic anemia. Splenectomy was eventually performed which achieved complete resolution of ascites, increase of hemoglobin concentration and abrogation of transfusion requirements.

关键词： Portosystemic shunt Hemosiderosis Splenectomy Intractable ascites Congenital dyserythropoietic anemia type Ⅱ

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