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

The clinical and ferrokinetic effects of escalating doses of subcutaneously administered recombinant human erythropoietin (rh-EPO) were studied in ten patients with myelodysplastic syndromes and severe transfusion-dependent anemia. Red blood cell transfusion requirements diminished in four patients, and one of the patients eventually became transfusion independent with an EPO-induced rise of Hb from 7.7 g/dl to 12.3 g/dl. Endogenous serum levels of EPO were significantly increased in all patients (100-5700 mU/ml), but three of four responders had a relatively low baseline level. The effective red cell iron turnover (RCIT) improved in two responding patients and even normalized in one patient. This increase in RCIT was accompanied with a decline in the ineffective red cell iron turnover (IIT). The other responding patients had a relatively preserved RCIT before EPO treatment. EPO therapy further increased the fraction of IIT in the latter patients. Red cell survival time did not increase during EPO therapy, even in the responding patients. One transient and one maintained increase in platelet count were observed. Disease progression with a sustained increase in blast cells in one patient and a transient elevation of blasts in another patient was seen. No other side effects of EPO therapy were observed. These results suggest that anemic MDS patients with low serum EPO levels and relatively spared effective erythropoiesis as measured by ferrokinetic studies may be the best candidates for treatment with recombinant human EPO.
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PMID:Recombinant human erythropoietin for the treatment of anemia in the myelodysplastic syndromes: a clinical and erythrokinetic assessment. 173 54

We have prospectively evaluated a regimen of mitozantrone and cytosine arabinoside (Ara-C) as first-line therapy in elderly patients with acute myeloid leukaemia (AML). One hundred and four patients with a median age of 68 (range 60-81) were studied, in whom 86 had de-novo AML, and 18 had preceding myelodysplasia or secondary AML. Complete remission was achieved in 64% of de-novo cases, in 28% of MDS/secondary cases, and in 58% overall. The incidence of early death within 28 d of chemotherapy was 11%. The median disease-free survival (DFS) was 11 months with an actuarial DFS of 15% at 43 months. The median overall survival was 9 months with an actuarial survival of 10% at 44 months. The incidence of non-haematological toxicity was acceptably low, and usually of mild to moderate severity. Quality of life was improved, or unchanged, in 90% of responders. We conclude that mitozantrone and ara-C is an effective and well-tolerated regimen which produces high remission rates in elderly patients with AML.
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PMID:Mitozantrone and cytosine arabinoside as first-line therapy in elderly patients with acute myeloid leukaemia. 158 Dec 25

12 patients with myelodysplastic syndromes were treated with recombinant human erythropoietin (r-epo). 5 patients had stable anemia, 78-92 g/l, and 7 were transfusion-dependent. In 11 patients, r-epo was given intravenously three times a week, with dose escalation after 4 and 8 wk if hemoglobin did not increase more than 15 g/l. The doses were 600, 1500 and 3000 U/kg bodyweight/wk. The 12th patient was treated subcutaneously with a dose of 560 U/kg/wk. 3 patients showed a significant response with an increase in hemoglobin of greater than or equal to 15 g/l. 2 of these had stable anemia before treatment and increased in hemoglobin from 87 to 116 g/l and from 80 to 99 g/l, respectively. The 3rd patient was transfusion-dependent and rose to a stable hemoglobin level between 76 and 80 g/l without transfusions. 2 patients showed a reduction of their transfusion need. Mean initial serum erythropoietin in the responding group was 366 U/l compared to 1049 among the non-responders (p = 0.367). Response was observed in 5/7 patients without bone marrow sideroblasts and in 0/5 patients with sideroblasts (p = 0.027). Erythropoietin seems to be an effective and well-tolerated treatment for a certain proportion of patients with MDS. A larger patient material might provide a model for predicting responses.
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PMID:Treatment of myelodysplastic syndromes with recombinant human erythropoietin. 176 Nov 22

The bone marrow smears of 18 confirmed cases of MDS were analyzed carefully for the presence of "hypergranular type III blasts", defined as more than 20 fine azurophil primary granules per cells. The concordance was close to 80% among 5 observers. Thirty-nine percent (7 cases) were reclassified as RAEB-t rather than RAEB. The presence of these hypergranular blasts was not suggestive of increased differentiation but rather leukemic cells. The reassignment of cases altered the median survival for the various subcategories, providing a clearer separation with the introduction of type III blasts than without utilizing these cells which were separated from the promyelocyte family. The introduction of this new blast cell definition in a larger series of patients is recommended to confirm these preliminary observations.
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PMID:Prognostic implication and characterization of the blast cell population in the myelodysplastic syndrome. 176 64

The prognostic value of the FAB classification, bone marrow histology, Bournemouth score, and chromosome findings was studied in 88 patients with primary myelodysplastic syndromes. The median survival for the whole group of patients was 22 months (RA 61.7 months, RARS 31.6 months, CMML 15.7 months, RAEB 10.3 months, and RAEBt 8.2 months). Chromosomal abnormalities were found in 37 of the 70 patients investigated (52%). Only the differences in survival between patients with complex versus normal karyotype were statistically significant (p = 0.02). The presence of small blastic cells, located away from the endosteal surface (abnormal localization of immature blasts or ALIP) appears to be a major prognostic factor in predicting the duration of survival and progression to ANLL, especially in the FAB subgroups RA and RARS. Median survival for the 22 ALIP- cases with RA/RARS was 65 months, compared with 31 months for the ALIP+ cases (p = 0.0006). Nine ALIP+ patients (53%) developed ANLL in contrast to 3 (13%) of the ALIP- cases (p = 0.008). By redefining ALIP and evaluating the number and characteristics of the accompanying cells, histological subtypes were distinguished correlating largely with the FAB subgroups. Our findings demonstrate the prognostic importance of bone marrow biopsy and quantifying the complexity of bone marrow chromosome changes. It should be helpful in evaluating current attempts to find effective treatment for patients with MDS.
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PMID:Update on the prognostic implication of morphology, histology, and karyotype in primary myelodysplastic syndromes. 179 66

This study examines the occurrence, subtype and possible significance of haemopoietic inhibitory T cells (HIT cells) in patients with myelodysplasia (n = 21), acute myeloblastic leukaemia (n = 8) and in normal subjects (n = 13) using an autostimulary CFU-GM stem cell assay. HIT cells were detected when colony numbers increased by 30% or more following T-lymphocyte subpopulation ablation. HIT cells occurred in 28% of the MDS patients, were always CD8-positive and never occurred in the normal subjects. Sequential studies in MDS patients with HIT cells and treated with low dose Ara-C showed no correlation between occurrence of these cells and clinical outcome. HIT cells appear to be a clinically irrelevant manifestation of the cell dysfunction associated with MDS.
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PMID:The occurrence subtype and significance of haemopoietic inhibitory T cells (HIT cells) in myelodysplasia: an in vitro study. 183 Jun 30

We analysed the course and clinical features of a series of refractory anaemias (RA, RAEB, RAEBt). We could not find evidence to support the hypothesis that these three MDS classes are inevitably subsequent events of a single disease. Therefore aggressive treatment of RA, aimed at avoiding its evolution to RAEB or RAEBt, does not seem justified.
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PMID:[Clinical course of refractory anemia. Study of a case series of 56 patients]. 183 3

We have conducted several phase I/II clinical studies in a total of 65 MDS patients utilizing recombinant human hematopoietic growth factors including GM-CSF, IL-3, and EPO. Twenty-seven patients with MDS were treated with either continuous i.v. infusion or single daily s.c. injection of rhGM-CSF at dosages from 15 micrograms/m2 to 1000 micrograms/m2. All of them exhibited white cell responses during the treatment cycles, but no sustained rise in reticulocytes or platelets was recorded. In four of the patients, all with > or = 15% blast cells in the bone marrow, the percentage of circulating blast cells increased during treatment with rhGM-CSF (at dosages of 500 micrograms/m2 and 1000 micrograms/m2, respectively), although no leukemic conversion occurred. Of 9 patients treated so far with rhIL-3 at single daily s.c. dosages of 60 micrograms/m2, all exhibited white cell responses; 8 exhibited significant improved platelet and reticulocyte counts. Nineteen further patients received rhEPO for a period of 14 weeks by s.c. (10,000 U five times weekly) or i.v. bolus administration (150-450 U/kg). None of these patients experienced an increase in white cell and platelet counts. A significant increase of the reticulocyte count was recorded in 3 patients only. Another strategy involves the recruitment of leukemic cells into the cell cycle by hematopoietic growth factors followed by treatment with cycle-specific cytostatic agents. Therefore in 10 patients administration of rhGM-CSF (250 g/m2/day x 14, s.c.) was combined with Ara-C treatment (20 mg/m2/day x 14; s.c.). Initial results of this pilot study available in 5 patients indicated that this approach may control leukemic cell proliferation and may increase number of mature myeloid cells in both bone marrow and peripheral blood. A similar approach utilizing rhIL-3 in conjunction with Ara-C is on-going.
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PMID:Clinical use of recombinant human hematopoietic growth factors (GM-CSF, IL-3, EPO) in patients with myelodysplastic syndrome. 184 32

A method was developed for concentrating infectious pancreatic necrosis virus from hatchery water using positively charged 1-MDS filters. The method consists of passing large volumes (Ca. 1001) of hatchery water through 1-MDS microporous filter followed by the elution of the adsorbed virus using a high pH buffer. The virus adsorbed efficiently to 1-MDS filters when the pH of the water was 5.5 and was eluted optimally with 3% beef extract solution (pH 10). This procedure permitted the processing of 100 1 of hatchery water which resulted in a 300-fold reduction in the volume of water and greater than 90% recovery of the seeded virus.
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PMID:Method for the concentration of infectious pancreatic necrosis virus from hatchery water. 186 6

Two simple statements can be given which reflect our helplessness with respect to myelodysplasia. MDS are rare in childhood, but features and course of disease resemble those in adults. JCMML should be considered as a pediatric subtype of MDS, but with a worse prognosis than CMML in adult patients.
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PMID:Myelodysplasia in childhood. 186 75


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