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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ferrokinetic measurement were performed in a total of 48 patients with myelodysplastic syndrome (MDS), 13 with hypoplastic MDS and 25 with aplastic anemia (AA). Forty seven % of patients with hypoplastic MDS progressed to acute leukemia, however none of the patients with AA progressed to acute leukemia. Sixty-nine% of the patients with MDS showed increased erythron transferrin uptake (ETU) caused by ineffective erythropoiesis. On the other hand, 85% of the patients with hypoplastic MDS and all patients with AA showed decreased ETU caused by reduced erythropoiesis. Positive correlation was observed between ETU and the erythroid precursor cells in the bone marrow. Bone marrow scintigraphy utilizing 99mTc-sulphur colloid showed peripheral expansion of active marrow in MDS patients and islands-like distribution in hypoplastic MDS and AA patients. Ferrokinetics and bone marrow scintigraphy demonstrated the difference between typical MDS and hypoplastic MDS. Hypoplastic MDS appears to be a distinct clinicopathologic entity.
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PMID:[Ferrokinetics and bone marrow scanning in patients with myelodysplastic syndrome, hypoplastic myelodysplastic syndrome and aplastic anemia]. 847 76

Anaemia is a common manifestation of juvenile rheumatoid arthritis (JCA). We have evaluated 26 JCA patients with anaemia and compared their laboratory parameters to those without anaemia. In the patients with anaemia, activation criteria such as erythrocyte sedimentation rate (ESR) and CRP were significantly higher than in those without anaemia. Anaemia was present in all systemic JCA patients and was present in 42% and 78% of the oligoarticular and polyarticular types, respectively. Serum iron levels and transferrin saturations were low in all, whereas serum iron-binding capacities of the patients were normal. Mean ferritin level was 249pg/l (range 8.46-1000pg/l). There was a significant correlation between ferritin levels and CRP and ESR (r = 0.48 and r = 0.55 respectively) (both p < 0.05). Epo levels were normal. Twelve (60%) of the bone marrow aspiration specimens stained positive for iron whereas 40% stained negative; there were also changes suggestive of myelodysplasia. Sideroblasts were also decreased in number. Thus, in these patients iron is not sufficiently transferred to the erythroid series and/or cannot be used by erythroblasts, accompanied by a possible absolute iron deficiency. Thus we suggest that the iron in JCA tends to be stored in the form of ferritin, not in an accessible form and impaired metabolism along with other factors are effective in the anaemia of JCA.
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PMID:Anaemia in juvenile chronic arthritis. 879 53

In this study the analytical performances of two recently introduced assays for soluble transferrin receptors in serum were tested. The Ramco transferrin assay was compared with the Eurogenetics assay. In a small clinical study serum samples from patients with anaemia of chronic disease, iron deficiency and myelodysplastic syndrome were analysed, as well as sera from healthy volunteers. The analytical performances of the Ramco assay were found to be acceptable. In the Eurogenetics test however, inter-assay imprecision and the end of run drift were unacceptably high. We were able to confirm that in patients with uncomplicated iron deficiency the concentration of soluble transferrin receptors is higher than in healthy volunteers. In cases of anaemia of chronic and inflammatory disease, the levels of soluble transferrin receptors in serum are slightly, but not significantly, higher than in normal subjects. Measurement of soluble transferrin receptors in serum provides a good differentiation between anaemia of chronic disease and iron deficiency.
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PMID:Analytical and clinical implications of soluble transferrin receptors in serum. 936 99

Ineffective erythropoiesis was evaluated in 100 studies in 87 patients with myelodysplastic syndromes (MDS) applying newly devised parameters based on ferroerythrokinetic (FEK) data including erythron transferrin uptake (ETU). The efficiency ratio (R-Ef) was calibrated with V-2, the second canonical discriminant variate which underwent axis rotation. Based on discriminated FEK patterns, as previously reported, the patients were classified into five types i.e. I-z, ineffective, H-z, hemolytic hyperplastic, O-z, hypoplastic, I-H-z and I-O-z, combination of these, groups. The proportion of I-z group was 48% and that of I-H-z and I-O-z groups was 30%. Significant correlations were obtained in I-z group between R-Ef and ETU (r = -0.628), unclear abnormality scores (R = 0.664) or ringed sideroblast scores (R = 0.742), scored by dummy variables regression. In the other groups, significant deviation from these correlations was observed, indicating that there is an obvious heterogeneity in MDS patients concerning association or dissociation of morphological abnormality, in addition to hyperplasia of erythroblasts, with or from ineffective erythropoiesis.
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PMID:[Heterogeneity of ineffective erythropoiesis in myelodysplastic syndromes evaluated by application of newly devised parameters based on ferroerythrokinetic data]. 902 54

The presence of transferrin receptors on erythroblasts in patients with iron deficiency, anaemia of chronic disease (ACD) and myelodysplastic syndrome (MDS) was studied by two-colour analysis on a flow cytometer. CD 71 was used to quantify the number of transferrin receptors and GLY-A to identify erythroblasts. In cases of iron deficiency, the number of transferrin receptors was increased on part of the erythroblasts thus facilitating iron uptake by the cells. In patients with ACD or MDS, a decrease of the number of transferrin receptors on erythroblasts was found. This leads to the conclusion that the ineffective response to iron therapy in cases of ACD and MSD can be explained by a decline of transferrin receptors on the red cells.
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PMID:The expression of transferrin receptors on erythroblasts in anaemia of chronic disease, myelodysplastic syndromes and iron deficiency. 906 6

Six patients with myelodysplastic syndromes (MDS) and two patients with chronic lymphocytic leukemia, all with severe anemia entered the study. Before treatment reasons of secondary anemia were excluded. Concentration of erythropoietin, iron, transferrin, ferritin were measured before, and in the second and the third month of the trial. A r-HuEpo dosage of 80 U/kg was administered intravenously three times weekly for a minimum of three months. Four patients finished the study. The increase in hemoglobin concentration by 6 g% was observed in one patient with MDS subtype RA. In three other patients who apart from r-HuEpo received chemotherapy transfusion requirements decreased by 90%. Together with increase in hemoglobin decrease in ferritin was observed. The correlation between r-HuEpo and endogenous erythropoietin and ferritin was defined.
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PMID:[Preliminary results of erythropoietin treatment of anemia in myelodysplastic syndromes and chronic lymphocytic leukemia]. 928 12

The expression of transferrin receptors on the cell membrane of erythroblasts was analysed with flow cytometry in patients with different forms of anaemia. At the same time the concentration of soluble transferrin receptors (sTfRs) was analysed in serum. It was shown that only in iron deficiency a high concentration of sTfRs in serum could be explained with an increased expression of transferrin receptors on the erythroblastic membrane. In anaemia of chronic disease and myelodysplasia a discrepancy between a low expression on the cell membrane and normal or elevated serum values was seen. From this study we conclude that the concentration of sTfRs in serum does not only depend on the expression of transferrin receptors on the erythroblasts but also on the erythroid activity.
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PMID:Relationship between soluble transferrin receptors in serum and membrane-bound transferrin receptors. 949 May 71

Effective management of early anaemia in the course of chronic renal insufficiency requires the following: (i) implementing an efficient diagnostic strategy to exclude common contributing factors; (ii) initiating epoetin therapy for the majority of patients; for and (iii) ensuring adequate iron supply erythropoiesis. Diagnostic inquiry is warranted whenever the haemoglobin concentration is below the normal range adjusted for age and gender. The most efficient diagnostic approach is to assume erythropoietin deficiency, exclude iron deficiency, and pursue further diagnostic tests only when red-cell indices are abnormal or when leukopenia or thrombocytopenia are also present. Macrocytosis should prompt an inquiry into alcoholism, B12 deficiency, or folate deficiency. Microcytosis suggests iron deficiency or thalassaemia. Associated cytopenias raise the possibility of alcohol toxicity, pernicious anaemia, malignancy, or myelodysplastic syndrome. Epoetin therapy is warranted whenever the haemoglobin concentration has fallen below 10.0 g/dl. To initiate therapy prior to dialysis, epoetin should be administered at an average dose of 100 IU/kg/week (80-120 IU/kg/week, 50-150 IU/kg/ week) by subcutaneous injection. Haemoglobin concentration should be monitored every 2 weeks and the epoetin dose adjusted by increments or decrements of 25% to maintain a rate of rise of haemoglobin concentration of 0.2-0.6 g/dl (0.3 0.6 g/dl/week, 0.2-0.5 g/dl/week). When the target range is achieved, the dose of epoetin should be continually adjusted to maintain a stable haemoglobin concentration. Transferrin saturation and ferritin concentration should be monitored monthly, and sufficient iron provided to maintain transferrin saturation above 20%. The lower the haemoglobin concentration, the greater the likelihood that future intravenous iron will be required. Oral iron supplements should be avoided, since they are costly, ineffective, and troublesome to patients. Finally, a blunted therapeutic response to epoetin therapy provides important diagnostic information and gnostic inquiry.
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PMID:Management of early renal anaemia: diagnostic work-up, iron therapy, epoetin therapy. 1103 56

Iron is essential for cell proliferation, heme synthesis, and a variety of cellular metabolic processes. In most cells, transferrin receptor-mediated endocytosis is a major pathway for cellular iron uptake. Recently, transferrin receptor 2 (TfR2), another receptor for transferrin, was cloned. High levels of expression of TfR2 messenger RNA (mRNA) occur in the liver, as well as in HepG2 (a hepatoma cell line) and K562 (an erythroid leukemia cell line). In this study, TfR2 mRNA expression was analyzed in hematological cell lines, normal erythroid cells at various stages of differentiation, and leukemia and preleukemia cells. High levels of TfR2 expression occurred in all of the erythroid cell lines that were examined. Erythroid-specific expression of TfR2 protein in bone marrow cells was confirmed by immunohistochemical staining. Expression of TfR2 mRNA was high in normal CD34(+) erythroid precursor cells, and levels decreased during erythroid differentiation in vitro. Levels of expression of TfR2-alpha mRNA were significantly higher in erythroleukemia (M6) marrow samples than in nonmalignant control marrow samples. In addition, relatively higher levels of TfR2-alpha mRNA expression occurred in some samples of myelodysplastic syndrome that had erythroid hyperplasia in bone marrow, acute myelogenous leukemia M1, M2, and chronic myelogenous leukemia. Expression profiles of normal members of the erythroid lineage suggest that TfR2-alpha may be a useful marker of early erythroid precursor cells. The clinical significance of TfR2-alpha expression in leukemia cells remains to be determined.
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PMID:Expression of transferrin receptor 2 in normal and neoplastic hematopoietic cells. 1167 42

Genotypic testing of nonselected patients with the myelodysplastic syndrome (MDS) for the C282Y and H63D mutations of the HFE gene responsible for hereditary hemochromatosis revealed a significantly increased frequency of these mutations when compared to healthy blood donors reflecting the average population. Among the 50 patients examined [26 refractory anemia (RA), 9 refractory anemia with ring sideroblasts (RARS), 2 refractory anemia with excess of blasts (RAEB) and 13 refractory anemia with excess of blasts in transformation (RAEB-t)] there were 24 heterozygotes (20 for H63D and 4 for C282Y), 1 homozygote for H63D and 1 compound heterozygote. The difference between the HFE-positive and HFE-negative MDS patients as regards initial serum iron and transferrin saturation was not significant. Inevitably the iron overload syndrome eventually develops in MDS patients due to intrinsic characteristics of the disease as well as an escalating need for blood transfusion therapy in the course of the disease. The high incidence rate of HFE gene mutations among MDS patients may also contribute to this vicious circle.
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PMID:High incidence of hemochromatosis gene mutations in the myelodysplastic syndrome: the Budapest Study on 50 patients. 1262 89


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