Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pancreatic islets of 36 autopsy cases with transfusional iron-overload were examined. Immunohistochemical and histochemical stainings were used to clarify the relationship between blood transfusion and iron deposition in the islet. Disease of the lymphohemopoietic system (leukemia, lymphoma, aplastic anemia) or liver (carcinoma and/or cirrhosis) accounted for 86.1% of the patients' main diagnosis. Sixteen of them had slight hemosiderin deposition (Group 1), twenty cases had severe hemosiderin deposition (Group 2). Another ten cases were used as controls (Group 3). The cases had a similar age distribution to Group 1 and 2, with neither blood transfusion nor hemosiderin deposition. The volume of blood transfusion was 6.1 +/- 3.6, 17.5 +/- 12.2 L for Groups 1 and 2, respectively. The plasma glucose was 137.8 +/- 54.4 and 170.6 +/- 108.4 mg/dL, respectively. Four cases in Group 1 and 14 cases in Group 2 had glycosuria. The number of islet cells with hemosiderin increased with the enlargement of transfusion volume (r = 0.664, P < 0.001). Plasma glucose also related with the percentage of hemosiderin positive islet cell (r = 0.386, P < 0.025). In severely iron-overloaded cases, hemosiderin was selectively deposited in B cells of the islet. It was concluded that large amounts of blood transfusions for non-congenital disease can induce selective hemosiderin deposition and impairment of pancreatic B cell that may result in hyperglycemia and diabetes mellitus of the patients.
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PMID:Selective iron deposition in pancreatic islet B cells of transfusional iron-overloaded autopsy cases. 802 61

A case of a 64-year-old Japanese woman who developed aplastic anemia and idiopathic thrombocytopenic purpura with antibody to platelet glycoprotein IIb/IIIa 4 years following a resection of malignant thymoma is reported. Bone marrow was hypocellular and ferrokinetics revealed the prolongation of the half-time of plasma iron disappearance and a decrease in red cell utilization, findings compatible with a diagnosis of aplastic anemia. The life span of platelets was markedly decreased to 3.07 h, and the test for antiplatelet glycoprotein IIb/IIIa antibody was positive. The patient's serum had a suppressive effect on the formation of colonies of burst-forming unit-erythroid and colony-forming unit-granulocyte using normal bone marrow cells. These results suggest that the aplastic anemia in this patient may have been induced by some suppressive activity in the serum. Splenectomy followed by an administration of cyclosporine effectively restored the peripheral blood count.
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PMID:Aplastic anemia and idiopathic thrombocytopenic purpura with antibody to platelet glycoprotein IIb/IIIa following resection of malignant thymoma. 823 73

To distinguish hypoplastic myelodysplastic syndrome (MDS) from aplastic anemia (AA), morphological abnormalities of bone marrow hematopoietic cells in 8 patients with MDS and 39 patients with AA were studied. All the patients with MDS and AA showed prolonged plasma iron disappearance time, (PIDT1/2) > 120 min. Five hundred erythroid and myeloid cells, as well as 20 megakaryocytes were counted. Dysplastic changes were defined if morphological changes were present in more than 1.0% cells with only one lineage, or in more than 0.6% cells with more than two lineages. Twenty six of 39 patients with AA showed morphological abnormalities. In MDS cases, morphological abnormalities were prominent in trilineage cells in some cases, in bilineage (erythroid and megakaryocytic or myeloid and megakaryocytic cells), in others, or solely in myeloid cells or in megakaryocytic cells in other cases. Morphological abnormalities seen solely in erythroid cells, especially those with segmented nuclei were considered to be less significant for the diagnosis of MDS. The findings were considered to be useful to distinguish hypoplastic MDS from AA.
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PMID:[A study of hypoplastic myelodysplastic syndrome]. 825 4

High renal uptake on bone marrow scan with indium-111 chloride is often shown in patients with bone marrow abnormality. We evaluated the renal uptake on bone marrow scan in 27 cases with aplastic anemia, 20 cases with myelodysplastic syndrome (MDS) and 10 cases with other diseases. The high renal uptake was observed in patients not only with aplastic anemia but also with MDS. The renal uptake correlated with blood transfusion units, unsaturated iron binding capacity (UIBC), blood pool imaging and bone marrow uptake. The renal uptake correlated with UIBC better than with the blood transfusion units. Following mechanism of the renal uptake is proposed that frequent blood transfusion makes low UIBC, and the low UIBC causes the failure to chelate indium with transferrin. The fast blood clearance of un-chelated indium via kidneys is followed. Hypoplastic bone marrow may also play an important role for the high renal uptake because all patients with the high renal uptake could not be explained by above mentioned mechanisms. Caution should be paid to the scans with the high renal uptake because both aplastic anemia and MDS patients show the high renal uptake.
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PMID:[Evaluation of renal uptake on 111InCl3 bone marrow scintigraphy in patients with aplastic anemia and myelodysplastic syndrome]. 831 88

With a newly developed short term enzyme linked immunosorbent assay kit (TOYOBO Co.), in which 2 kinds of anti-EPO monoclonal antibodies were used, we assayed EPO concentration in sera from patients with renal failure and hematological disorders. In this report, the EPO data were analysed in relation to serum iron concentrations, with ferritin and UIBC. In the patients with renal failure, there was no significant correlation between EPO concentration and serum iron, ferritin, nor UIBC concentration. On the other hand, in the patients with hematological disorders, there were two types. One was in patients with iron deficiency anemia, whose serum EPO was negatively correlated to serum iron (r = -0.64) and ferritin (r = -0.59), but positively related to UIBC (r = 0.27). The another was the pattern in patients with aplastic anemia, leukemia and MDS, whose serum EPO positively correlated to iron and ferritin but negatively correlated to UIBC. In the patients with aplastic anemia serum EPO had good correlation to serum iron (r = 0.62), ferritin (r = 0.60) and UIBC (r = -0.46). The relationship of EPO to iron in the patients with leukemia (r = 0.54), and EPO to ferritin in the patients with MDS (r = 0.42) show significantly positive correlation coefficient.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Assay of erythropoietin in serum with short term enzyme linked immunosorbent assay method--the clinical significance: Part 2--:Relation to serum iron, UIBC and ferritin in renal failure and hematological disorders]. 835 May 9

We report a case of aplastic anemia complicated with secondary hemochromatosis after allogenic bone marrow transplantation (BMT). A 29-year-old man was diagnosed as having aplastic anemia at the age of 8. At the age of 28, BMT was performed from his HLA-identical sister. Total volume of blood transfusion before BMT was about 28,000 ml, and in three months after BMT was 8,000 ml. The transplantation was successful, but one month after BMT, dry eyes, skin pigmentation and hepatomegaly appeared. Serum bile duct enzymes and ferritin also increased remarkably. Moreover after thirteen months, glucose tolerance impaired seriously. Abdominal computed tomography (CT) revealed atrophic pancreas and an increased CT density in the liver and the tail of the pancreas. A large amount of iron deposition were also found in liver and stomach biopsy specimens. We concluded that diabetes mellitus was due to secondary hemochtomatosis in the present case. There is a possibility that tissue damage due to iron deposits may have been accelerated through BMT in this patient with a history of many blood transfusions.
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PMID:[Aplastic anemia complicated with secondary hemochromatosis after allogenic bone marrow transplantation]. 853 29

Nineteen children and adolescents receiving repeated transfusions and subcutaneous desferrioxamine treatment were investigated in an attempt to quantitate iron overload non-invasively. Before patients were started on desferrioxamine individual relationships were correlated for 12 to 36 months between transfused iron, absorbed iron estimated gastrointestinally, and increasing serum ferritin concentrations. Patients with inflammation, increased liver enzymes, or haemolysis were excluded from analysis. The relationship between the variables could be described by a logarithmic regression curve (y = transfused iron [plus eventually gastrointestinally absorbed iron] = iron overload = a+b log [x = serum ferritin]) for each individual patient. All patients showed close correlation (R2) between x and y (median R2 of 0.909, 0.98, and 0.92 in thalassaemia, aplastic anaemia, and sickle cell anaemia patients, respectively). When started on desferrioxamine, current serum ferritin concentrations were used to derive the iron overload from each individual regression curve. The derived estimated iron overload ranged from 0.6 g to 31 g. Left ventricular dilatation was observed in three patients with beta thalassaemia and in one patient with aplastic anaemia with median iron overload of 20.7 (14.1-31.3) g and 24.0 g respectively. Hypothyroidism was found in four patients with beta thalassaemia and one patient with aplastic anaemia with iron overload between 14.7 (6.8 and 26.1) g and 15.1 g respectively. Human growth hormone deficiency was detected in three patients with beta thalassaemia with an iron overload of 4.2 (3.5-6.8) g; all three patients had excellent desferrioxamine compliance.
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PMID:Logarithmic quantitation model using serum ferritin to estimate iron overload in secondary haemochromatosis. 866 33

Severe aplastic anemia (SAA) is a disease associated with high mortality. For young patients with HLA identical siblings, allogeneic bone marrow transplantation (BMT) offers the best chance of cure. Favourable results have also been reported using immunosuppressive therapy (IST). Transplantation is usually favoured for patients below 45 years of age. We report our experience of 11 allogeneic and one syngeneic BMT for adult Chinese patients with SAA, over a 4-year period from 1991 to 1995. Ten of the 12 (83 per cent) patients had received and failed prior IST including anti-thymocyte globulin (ATG) before being referred for BMT. Neutrophil and platelet engraftment was successful in 11 of them (92 per cent) and nine were completely transfusion independent after transplantation. Their overall 3-year survival was 67 per cent. The compromised overall result was due to a number of cases transplanted after a long time delay. No patient transplanted beyond 3 years from the initial time of diagnosis of SAA achieved long-term marrow engraftment, and they all eventually succumbed. On univariate analysis, a longer time delay and hence a larger amount of blood products exposure, were highly significantly statistically associated with inferior marrow engraftment and patient survival. Other factors including age, iron status, infused cell dose and the conditioning protocol were not found to significantly affect engraftment and survival. Graft versus host disease was clinically mild or absent in most patients. This may be related to ethnicity or previous ATG exposure. In conclusion, early allogeneic BMT was a safe and effective treatment in our small series of patients with SAA failing IST.
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PMID:Allogeneic bone marrow transplantation for severe aplastic anemia: the Hong Kong scenario. 1006 11

By ways of HPLC and RIA, multiple laboratory parameters were determined simultaneously in 84 patients of iron--deficiency anemia (IDA) or chronic aplastic anemia (CAA) with Liver-Blood Deficiency Syndrome (LBDS) diagnosed by differentiation of TCM. The results showed that LBDS had some pathophysiological characteristics such as decreased function of sympathicus and adrenal medulla axis, increasing of L-EK, lower energy metabolism of erythrocyte, lower rate of body metabolism, sodium and water imbalance, and abnormalities in hemorrheology and erythrocyte deformability. It is suggested that the patients with LBDS are in a state of protective compensation in blood volume regulation.
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PMID:[Preliminary study on pathophysiological basis of liver-blood deficiency syndrome]. 1007 22

Recently the number of long-term survivors of aplastic anaemia has increased, with some of these cases evolving into myelodysplastic syndrome (MDS). Because it is difficult to discriminate between aplastic anaemia and hypoplastic MDS, it is unknown whether these patients have had MDS from the time of diagnosis of aplastic anaemia. Presence of ringed sideroblasts on an iron-stained bone marrow smear is a characteristic of some cases of MDS. Amorphous electron-dense deposits in the mitochondrial matrices of erythroid precursors observed with an electron microscope show ringed sideroblasts, and detection of this mitochondrial pathology is useful for confirming the presence of ringed sideroblasts because this mitochondrial pathology can be found not only in erythroblasts but also in reticulocytes, which is particularly useful in cases in which few erythroblasts are found. We found this mitochondrial pathology in two of nine children who had an initial diagnosis of aplastic anaemia and in three children with hypoplastic MDS. It is unknown at present whether the first two children had aplastic anaemia or hypoplastic MDS. Our results warrant further studies on more patients to confirm the significance of amorphous electron-dense deposits in the mitochondrial matrices of erythroid precursors.
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PMID:Significance of electron-dense deposits in the mitochondrial matrix of erythroid precursors in aplastic anaemia and myelodysplastic syndrome. 1023 78


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