1/11. Severe congenital dyserythropoietic anaemia type I: prenatal management, transfusion support and alpha-interferon therapy.We report a case of congenital dyserythropoietic anaemia, type I, with severe pre- and postnatal manifestations. Exchange transfusions were required for fetal anaemia (3.5 g/dl) at 28 and 30 weeks of gestation. Transfusions were administered at birth (Caesarean section at week 35) and at regular intervals thereafter. At 14 months, alpha-interferon therapy was initiated (106 units three times a week). This resulted in stabilization of the haemoglobin at or above 11 g/dl and a reduction in the percentage of erythroblasts with ultrastructurally abnormal heterochromatin. After 9 months, the dose of alpha-interferon was decreased to 106 units twice a week. No relapse of anaemia was noted during an additional 4 months of follow-up.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
2/11. Hyporegenerative anemia associated with Rh hemolytic disease: treatment failure of recombinant erythropoietin.A postnatal hyporegenerative anemia may complicate Rh hemolytic disease. Intramedullary hemolysis, bone marrow suppression, and erythropoietin deficiency have been implicated etiologically. Treatment with recombinant erythropoietin (r-EPO) has yielded encouraging preliminary results. The authors describe an infant with rh isoimmunization who developed severe hyporegenerative anemia unresponsive to a 5-week course of r-EPO. Two additional doses at 12 weeks resulted in brisk reticulocytosis, coinciding with a 16-fold decline in the anti-Rh(D) antibody titer. Thus, treatment with r-EPO may be ineffective when anti-Rh(D) antibody titers are high. The authors also show that erythropoietin deficiency in hyporegenerative anemia is not as frequent and severe as originally thought.- - - - - - - - - - ranking = 0.19062781054447keywords = hemolytic (Clic here for more details about this article) |
3/11. Allogeneic bone marrow transplantation for severe post-splenectomy thrombophilic state in leaky red cell membrane haemolytic anaemia of the stomatocytosis class.The tendency for thrombosis to occur if haemolysis persists after splenectomy is especially marked in "hereditary stomatocytosis", in which the red cell membrane "leaks" Na and K. A 21-year-old woman, who was splenectomized in childhood for a congenital haemolytic state, presented with major pulmonary embolism that recurred despite anticoagulation. Tests showed a significant cation leak with a "shallow-slope" abnormality in temperature dependence. Allogeneic bone marrow transplantation caused the thrombophilic state to cease and subsequently anticoagulation was stopped without recurrence of thromboembolism. However, she died 9 months after transplantation: iron overload, intensified by the transfusion demands of the transplant, was a major factor.- - - - - - - - - - ranking = 0.57142857142857keywords = anaemia (Clic here for more details about this article) |
4/11. Iron chelation therapy in aceruloplasminaemia: study of a patient with a novel missense mutation.We describe a novel missense mutation of ceruloplasmin in a patient with aceruloplasminaemia causing the replacement of a neutral amino acid (phenylalanine) with a polar one (serine) at position 198, probably leading to abnormal folding and secretion of the protein. The patient showed mild microcytic anaemia, mild hepatic iron overload, and marked brain iron overload. Six months of therapy with deferiprone was ineffective in removing iron from the tissues. deferoxamine was more efficient in removing excess iron from the liver but aggravated the disease related anaemia. After more than one year of chelation treatment, the brain magnetic resonance imaging signal did not change. overall, these findings indicate that treatment of iron overload in aceruloplasminaemia is a difficult challenge and that new iron chelators, more efficient in crossing the blood-brain barrier, are needed.- - - - - - - - - - ranking = 0.28571428571429keywords = anaemia (Clic here for more details about this article) |
5/11. Unusual splenic sinusoidal iron overload in sickle cell/haemoglobin D-Punjab disease.Sickle cell/haemoglobin D-Punjab disease is a disorder with similar clinical features to sickle cell anaemia. This report describes the case of an 11 year old boy with this disease who was treated with regular transfusions from infancy. He underwent splenectomy at the age of 10 years for hypersplenism. histology of the spleen revealed a striking pattern of heavy sinusoidal endothelial iron loading, with only moderate uptake by macrophages. Possible explanations for this unusual distribution of iron include phagocytosis of sickled erythrocytes by sinusoidal endothelial cells or direct endothelial iron uptake via transferrin receptors. Transfusion programmes ameliorate the symptoms of sickle cell disease but the dangers of iron overload should always be remembered.- - - - - - - - - - ranking = 0.14285714285714keywords = anaemia (Clic here for more details about this article) |
6/11. Desferrioxamine related maculopathy: a case report.Desferrioxamine is used for the treatment of chronic iron overload, acute iron poisoning, and certain anaemias. Ocular toxicity secondary to prolonged treatment with desferrioxamine may result in night blindness, visual field constriction, cataract, pigmentary retinopathy and optic neuropathy. To avoid such complications an ophthalmic screening has been suggested for patients taking desferrioxamine. We report an 81-year-old patient who developed irreversible ocular toxicity despite undergoing ophthalmic screening.- - - - - - - - - - ranking = 0.14285714285714keywords = anaemia (Clic here for more details about this article) |
7/11. Long-term alpha interferon treatment is effective on anaemia and significantly reduces iron overload in congenital dyserythropoiesis type I.Interferon has been shown to be an effective treatment of congenital dyserythropoiesis type I (CDA-I), but the optimal dose and the feasibility of this treatment remains to be determined. Here, in a 9-yr follow-up of a single patient, we show that interferon remains active during such a long period. The optimal dose of conventional alpha interferon could be evaluated at 2 million units twice a week. Pegylated interferon could be used as well at a dose of 30 microg/wk. During interferon treatment, serum and erythrocyte ferritin levels decreased progressively, and remained inversely correlated with haemoglobin levels. On repeated liver biopsies, iron overload could be normalized. Low dose interferon is a long-term treatment of CDA-I, and allows a significant decrease in iron overload, that could be interesting even in patients who are only moderately anaemic.- - - - - - - - - - ranking = 0.57142857142857keywords = anaemia (Clic here for more details about this article) |
8/11. Autoimmune hemolytic anemia: magnetic resonance findings.Idiopathic autoimmune hemolytic anemia (AIHA) is a rare hematological disease typically characterized by extracellular hemolysis. An unusual case of AIHA with interesting magnetic resonance imaging (MRI) appearances is presented. Possible explanations for the MRI findings are discussed.- - - - - - - - - - ranking = 0.19062781054447keywords = hemolytic (Clic here for more details about this article) |
9/11. liver cirrhosis as a consequence of iron overload caused by hereditary nonspherocytic hemolytic anemia.Nonspherocytic hereditary anemias are occasionally accompanied by significant iron overload but the significance for the development of chronic liver disease is not clear. We described two cases of patients with chronic liver disease and severe iron overload due to chronic hereditary hemolysis. Both patients have had signs of liver cirrhosis and severe hemolysis since childhood. A hereditary pyruvate kinase deficiency (PKD) was discovered as the underlying reason for the hemolysis. Sequencing of the pyruvate kinase gene showed a mutation within exon 11. Liver histology in both patients revealed cirrhosis and a severe iron overload but primary hemochromatosis was excluded by HFE-gene analysis. An iron reduction therapy with desferrioxamine led to significant decrease of serum ferritin and sustained clinical improvement. PKD-induced hemolysis may cause severe iron overload even in the absence of HFE-genotype abnormalities. This secondary iron overload can lead to chronic liver disease and cirrhosis. Therefore, the iron metabolism of PKD patients has to be closely monitored and iron overload should be consequently treated.- - - - - - - - - - ranking = 0.15250224843558keywords = hemolytic (Clic here for more details about this article) |
10/11. A case of rhesus hemolytic disease with hemophagocytosis and severe iron overload due to multiple transfusions.BACKGROUND: A newborn with cholestatic hepatic disease and hemophagocytic lymphohistiocytosis due to rhesus hemolytic disease (RHD) is reported. observation: A 34 weeks' gestation baby with RHD, who had received multiple intrauterine transfusions (IUT), developed cholestatic hepatic disease and secondary hemophagocytic lymphohistiocytosis (HLH). Her serum ferritin level increased to 5,527 ng/mL, and liver biopsy showed severe iron overload. Treatment with intravenous desferrioxamine resulted in a marked decrease in serum ferritin levels and normalization of liver function CONCLUSION: We suggest that patients who have undergone IUT be evaluated for hyperferritinemia. If hyperferritinemia is noted, chelation therapy should be considered. As another rare finding, HLH can complicate the course of RHD.- - - - - - - - - - ranking = 0.19062781054447keywords = hemolytic (Clic here for more details about this article) |
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