1/28. Juvenile hemochromatosis associated with B-thalassemia treated by phlebotomy and recombinant human erythropoietin.Juvenile hemochromatosis is a rare genetic disorder that causes iron overload. Clinical complications, which include liver cirrhosis, heart failure, hypogonadotropic hypogonadism and diabetes, appear earlier and are more severe than in HFE-related hemochromatosis. This disorder, therefore, requires an aggressive therapeutic approach to achieve iron depletion. We report here the case of a young Italian female with juvenile hemochromatosis who was unable to tolerate frequent phlebotomy because of coexistent ss-thalassemia trait. The patient was successfully iron-depleted by combining phlebotomy with recombinant human erythropoietin.- - - - - - - - - - ranking = 1keywords = iron overload, overload (Clic here for more details about this article) |
2/28. Complete heart block in thalassemia major: a case report.Cardiac complications of iron overload are the most common cause of death in patients with thalassemia major. These complications include recurrent pericarditis, refractory congestive heart failure and rhythm disorders. The usual rhythm disturbances are supraventricular or ventricular premature contractions and first-or second-degree heart block. Complete heart block is a very rare complication of thalassemia major. Herein, we report a case of complete heart block with thalassemia major. The patient also had serious congestive heart failure. Management of the heart block with pacemaker brought no clinical improvement, and she died in the second month of hospitalization.- - - - - - - - - - ranking = 1keywords = iron overload, overload (Clic here for more details about this article) |
3/28. Liver dysfunction and steatosis in familial hypobetalipoproteinemia.A 32-year-old man presented with increases in serum alanine aminotransferase activity, iron concentration, and transferrin saturation, suggestive of hepatic dysfunction and iron overload. In addition, he had unusually low plasma concentrations of LDL-cholesterol and apolipoprotein (apo) B. Hepatic ultrasonography was consistent with fatty liver. On liver biopsy, marked steatosis and moderate to marked iron deposition were observed. The patient was found to carry the HFE C282Y and H63D mutations, which are associated with hereditary hemochromatosis, and the alpha(1)-antitrypsin PiZ variant. An immunoblot of plasma for apoB showed the presence of a truncated apoB species, indicative of familial hypobetalipoproteinemia. dna sequence analysis revealed that the patient was heterozygous for the apoB-80.5 (c.11040T>G) mutation. This unique case shows an unusual combination of underlying disorders that could all be contributing to liver dysfunction and fatty liver.- - - - - - - - - - ranking = 1keywords = iron overload, overload (Clic here for more details about this article) |
4/28. A fatal case of disseminated zygomycosis associated with the use of blood glucose self-monitoring equipment.We report a fatal case of disseminated zygomycosis due to cunninghamella bertholletiae in a 68-year-old man with myelodysplasia and type II diabetes mellitus, receiving desferrioxamine therapy for iron overload secondary to multiple transfusions. It is thought that he acquired the infection through the use of blood glucose self-monitoring equipment.- - - - - - - - - - ranking = 1keywords = iron overload, overload (Clic here for more details about this article) |
5/28. Transfusional hemosiderosis and combined chelation therapy in sickle thalassemia.Although the indications for transfusions in sickle cell syndromes are well listed, and chronic transfusion has become practicable since the recent advances in chelation therapy have essentially eliminated the risk of secondary iron overload, multi-transfused, non-compliant to long-term chelation therapy patients confront the complication of iron overload and secondary hemosiderosis. In thalassemia major patients, combined therapy with desferrioxamine and deferiprone has maximized tissue iron removal and may reduce the overall occurrence of hemosiderotic heart failure. Despite this, safety and contradictions of chelating agents are still controversial. The aim of this report is to present the results of this combination in a long-term transfused sickle beta-thalassemic patient suffering from severe heart failure and liver dysfunction.- - - - - - - - - - ranking = 2keywords = iron overload, overload (Clic here for more details about this article) |
6/28. Hepatic adenomatosis associated with hormone replacement therapy and hemosiderosis: a case report.We have reported a case of hepatic adenomatosis associated with hormone replacement therapy (estrogen and progesterone) and hemosiderosis caused by excessive blood transfusion for the treatment of chronic myeloid leukemia. A 34-year-old woman was found to have several hepatic tumors on a routine medical examination. The general condition was good. Laboratory studies showed iron overload. Abdominal computed tomography and selective hepatic angiography showed several hypervascular tumors in the right lobe of the liver (up to 20 mm in diameter). Since hepatocellular carcinoma could not be ruled out, subsegmental hepatectomy was performed. Histopathological examination of the surgical specimen showed hepatic adenomatosis with hemosiderosis. Both hormone replacement therapy and iron overload could be the cause of hepatic adenomatosis.- - - - - - - - - - ranking = 2keywords = iron overload, overload (Clic here for more details about this article) |
7/28. Successful recovery of acute hemosiderotic heart failure in beta-thalassemia major treated with a combined regimen of desferrioxamine and deferiprone.We report the case of a 25-years-old male with beta-thalassemia major who developed acute heart failure, with severe systolic dysfunction, resulting from iron overload. Combined iron chelation with desferrioxamine and deferiprone together with standard cardiological treatment induced prompt and complete restoration of the cardiac function.- - - - - - - - - - ranking = 1keywords = iron overload, overload (Clic here for more details about this article) |
8/28. Correction of serious iron overload in a chronic hemodialysis patient by recombinant human erythropoietin and removal of red blood cells: confirmation by follow-up liver biopsy.A chronic hemodialysis case, a 46-year-old woman with secondary hemosiderosis induced by parenteral iron and blood transfusion due to a refractory anemia, was effectively treated with recombinant human erythropoietin and the removal of red blood cells. The cumulative dose of the iron removed was 5,712 mg. plasma ferritin decreased from 8,290 to 2,203 micrograms/l during 18 months. Concomitantly, liver histology performed before and after the therapy revealed a prominent regression of the deposited iron.- - - - - - - - - - ranking = 4keywords = iron overload, overload (Clic here for more details about this article) |
9/28. Congenital dyserythropoietic anaemia (type II) presenting with haemosiderosis.A 39-year-old female with type II congenital dyserythropoiesis presented with iron overload. The clinical and haematologic features were an anaemia of variable severity, splenomegaly, numerous bizarre and binucleate normoblasts in the bone marrow, with prominent submembranous cisternae in the late forms, a positive Ham's acid lysis test and aberrant expression of the I and i red cell antigens. The iron overload resulted from gross ineffective erythropoiesis, with accelerated plasma iron turnover and increased absorption aggravated by inappropriate replacement therapy for past episodes of anaemia.- - - - - - - - - - ranking = 2keywords = iron overload, overload (Clic here for more details about this article) |
10/28. Hepatic iron overload in renal transplant recipients: ultrastructural observations.This study describes the ultrastructural features of hemosiderosis occurring in five transplant recipients. Iron deposits in the form of homogeneous and heterogeneous siderosomes (iron-laden lysosomes) were found within the hepatocytes and macrophages in areas of fibrosis, and to a lesser degree within kupffer cells and bile duct epithelium. Disruption of siderosomal membranes with extrusion of ferritin particles into the cytosol was demonstrated in these specimens and may be significant in the pathogenesis of hepatocellular injury.- - - - - - - - - - ranking = 4keywords = iron overload, overload (Clic here for more details about this article) |
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