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

Erythroid and myeloid colonies were grown from the bone marrow of 81 patients with myelodysplasia and the median number of colonies correlated with the FAB classification and Bournemouth score. CFU-GM were increased in CMML compared to RAEB and RAEBt. BFU-E were higher in RA than in the other FAB subgroups. Patients with a high Bournemouth score had poorer CFU-GM and BFU-E growth than those with a low score.
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PMID:Correlation of bone marrow colony growth in the myelodysplastic syndromes with the FAB classification and the Bournemouth score. 279 88

Serum concentration of erythropoietin (Epo) has been measured by radioimmunoassay in 46 patients with myelodysplastic syndromes. There is an overall inverse relationship between the level of Epo and the degree of anaemia but a wide range of Epo response between patients with similar haemoglobin concentrations. No differences were found between the different FAB groups, but the highest Epo levels were found in those patients with erythroid hypoplasia in the bone marrow. It is suggested that the intensity of erythroid activity in the marrow, as well as the degree of anaemia, may be a factor determining serum Epo concentration.
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PMID:Circulating erythropoietin in patients with myelodysplastic syndromes. 280 75

The authors present a cytological study of 200 cases of myelodysplastic syndromes (MDS) [corrected], classified according to the FAB cooperative group. This analysis involves the conventional parameters of the peripheral blood and bone marrow differential counts, the systematic recording of signs of dysmyelopoiesis on the erythroblastic [corrected], granulocytic and megacaryocytic lineages and the assessment of blood granulocytes myeloperoxidase (MPO). On the outside of the typical acquired idiopathic sideroblastic anaemia (severe, isolated anaemia associated with intense dyserythropoiesis), the diagnosis of MDS requires very astute cytological interpretation: indeed, when the dysgranulopoiesis is pronounced, determination of peripheral blood and bone marrow differential count is especially difficult. Conversely when the dysmyelopoiesis is slight, a systematic search of morphology signs [corrected] of its presence must be done and associated with functional studies such as MPO activity technically easy to realize.
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PMID:[Cytological diagnosis of myelodysplastic syndromes. Apropos of 200 cases]. 282 45

This thesis is a survey of nine previously published articles on MPO deficient PMN. The incidences in leukaemia and allied disorders of the presence of this abnormal subpopulation of mature neutrophils and the relationship to clinical course in AML, susceptibility to infections in AML, FAB classification in AML and MDS, cytogenetically defined aberrations in MDS and morphometrical characteristics were investigated. The aims of the studies were to examine the diagnostic as well as the prognostic value of the parameter, to examine the usefulness of the parameter as an predictive indicator of CR and relapse in AML and to examine the concept that MPO deficient PMN may originate from leukaemic precursors. MPO deficient PMN were found to occur in a minor number (less than 4% of the total number of PMN) in normal humans and the incidences of an abnormal number (greater than 4%) were found to be about 40% in AML (I, II, III, IV, VIII), 60% in CML (I, VII), 30% in MPD other than CML (VII) and 30% in MDS (V). The highest incidences in AML were found in the FAB subtypes possessing the most myeloid differentiation potential i.e. FAB M2 and FAB M4 (IV). In ALL, CLL, HCL, Hodgkin's disease, anaemia not related to leukaemia and leukaemoid reactions the incidences all were 0% (I, unpublished data). The abnormal MPO deficient PMN subpopulation, if present, disappeared when CR was achieved and reappeared when relapse eventually was developed (II, VIII). In both situations serial determinations showed that the change occurred before the usual routine blood examinations predicted CR and relapse; several days and several months prior, respectively (VIII). The probability of obtaining CR was lower in the AML patients with the abnormal subpopulation and the risk of developing relapse higher than in AML patients without the anomaly (II, VIII). These differences were not statistically significant, however. AML patients, showing an increased number of MPO deficient PMN, revealed a statistically significant increased susceptibility to infections (P less than 0.01) during the preremission phase accounting for 18% to 67% of the total number of infections in this period (III). This increase was positively correlated to the extent of the anomaly (P less than 0.002). The spontaneous occurrence of a subpopulation of MPO deficient PMN in MDS went together with a simultaneous progression in cytogenetically determined clonal chromosomal aberrations and were related to progression in FAB subtype as well (VI). Morphometrically MPO deficient PMN were characterized by a decreased total cell size and an increased nucleus size of the projected images (IX).(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Myeloperoxidase deficient polymorphonuclear leucocytes in leukaemia and allied disorders. 285 15

In 45 cases of primary myelodysplastic syndrome; 16 refractory anaemia (RA), 11 RA with ring sideroblasts (RA+), 13 RA with excess of blasts (RAEB), 5 chronic myelomonocytic leukaemia (CMML), the relations between myeloperoxidase (MPO) activity in polymorphonuclear leucocytes (PMN), neutrophil alkaline phosphatase (NAP) activity, absolute number of PMN and thrombocytopenia were investigated. 11 patients (26%) showed abnormal numbers (greater than 4%) of MPO-deficient PMN and 27 (75%) showed abnormal NAP activity (NAP score; greater than 134.0, less than 15.0), mostly decreased. No significant correlations between MPO activity and NAP activity were demonstrated, nor were any significant correlations found with the other parameters investigated. The FAB-subtypes, RAEB and CMML, showed a significant correlation to thrombocytopenia (p = 0.028) and to pancytopenia (p = 0.024). The findings may support the view that at least some of the myelodysplastic syndromes may be fundamentally the same disease as acute myeloid leukaemia.
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PMID:Myeloperoxidase-deficient polymorphonuclear leucocytes. (V): Relation to FAB-classification and neutrophil alkaline phosphatase activity in primary myelodysplastic syndromes. 299 23

Relations between cytogenetic status, FAB-classification and an abnormal subpopulation of myeloperoxidase (MPO)-deficient polymorphonuclears (PMN) in 45 patients with myelodysplastic syndrome (MDS) are reported. Clonal abnormalities were demonstrated in 85% of the patients, with a lower incidence in the RA+ group (refractory anaemia with ring sideroblasts) compared to the others (p = 0.004). In 12 patients a spontaneous progression in cytogenetic aberrations occurred and in 7 of these (60%) a simultaneous progression in FAB-subtype was seen. The appearance of MPO-deficient PMNs was observed in 6 of these patients (55%). A progression in FAB-subtype was noted in further 4 patients and 2 additional patients developed MPO-deficient PMNs. Only one sufficient cytogenetic investigation was available in these patients. Thus 100% of the fully studied patients showed progression in cytogenetic abnormalities when a progression in FAB-subtype or a development of MPO-deficient PMNs was seen. 3 (49%) of the 8 patients developing MPO-deficient PMNs too showed a progression in FAB-subtype. Although no significant correlation to specific categories of structural aberrations or abnormalities in specific chromosome pairs could be demonstrated, clonal cytogenetic aberrations seem to be involved when the disease progresses and when MPO-deficient PMN develop.
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PMID:Myeloperoxidase-deficient polymorphonuclear leucocytes (VI): Relation to cytogenetic abnormalities in primary myelodysplastic syndromes. 300 22

Dihydrofolate reductase (FH2-R) was studied cytochemically in the bone marrow erythroblasts of 20 normal controls and 46 patients with myelodysplastic syndromes (MDSs) classified according to FAB, prior to therapy. The reaction product was quantified for the same samples with a Vickers M86 microdensitometer. The enzyme activity progressively decreased during the normal differentiation of the erythroid cells and persisted at high levels in MDS cells. The high level of FH2-R may be related to the malignant transformation of the cells, or to increased compensatory erythropoietic activity of ineffective erythropoiesis, or to both.
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PMID:Qualitative and quantitative study of dihydrofolate reductase in myelodysplastic syndromes. 313

Based on a 6 1/2-year study of 284 consecutive adult patients with primary myelodysplastic syndrome (MDS) and and acute myelogenous leukaemia (AML), we have found that refined chromosome analysis can be used as an independent prognostic indicator in the great majority of patients with MDS and AML. In MDS, the FAB subtype was also found to have prognostic value and this was enhanced when the chromosomal findings were taken into consideration. In AML, the age of the patient correlated more closely with the chromosomal changes in predicting prognosis in most patients than did the FAB classification. Previously we reported that refined chromosome analysis of bone marrow specimens from 161 adult patients with primary or non-therapy related MDS and AML identified three prognostic chromosomal categories in each disease, representing 40% of all patients (Yunis et al, 1984, 1986). By extending our study to 284 patients, as well as a longer follow-up, it was possible to determine the prognostic implications of two additional chromosomal categories in MDS and five in AML. Since 73% of all patients are now represented in well-defined chromosomal subgroups with prognostic significance, refined chromosome analysis emerges as a tool that could have considerable impact in protocols.
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PMID:Refined chromosome study helps define prognostic subgroups in most patients with primary myelodysplastic syndrome and acute myelogenous leukaemia. 316 79

Leukaemic clonogenic cells, capable of forming colonies of blast cells in an in-vitro assay, were examined for surface antigen expression using a panel of monoclonal antibodies (Mabs) to stem cell and myeloid differentiation antigens in nine cases of acute myeloid leukaemia (AML) and four cases of chronic myeloid leukaemia in myeloid blast crisis (CML-MBC). Clonogenic cells were found to be most frequently positive with anti-HLA-DR (positive in 100% cases) and RFB-1 (71%) Mabs, with significant reactivity also being seen with CD-33 (69%) and CD-13 (61%) myeloid specific antibodies. CD-11b and CD-15 antigens, expressed predominantly on mature leucocytes, were not significantly expressed on the clonogenic population. Interestingly, the CD-34 antigen, detected by MY-10 Mab on normal myeloid progenitor cells, was demonstrated on the clonogenic fraction of only one of seven cases tested. A discrepancy between antigen expression of clonogenic cells and immunophenotype of the total leukaemic population was frequently seen, with "early" markers (CD-33, HLA-DR, RFB-1) expressed on a higher proportion of the clonogenic fraction than the overall population, while the converse was the case for the "later" marker, CD-11b. Based on the known normal distribution of differentiation antigens, particularly the CD-13 antigen, cases could be ranked according to clonogenic phenotype into immature (CD-13- HLA-DR+ CD-33+ or CD-33-; five cases), and mature (CD-13+ HLA-DR+ CD-33+; eight cases), levels. However, there was no correlation between these maturation levels and the morphology according to the FAB classification. Of note, the mature group included three CML-MBC, as well as two AML cases with a history of myelodysplasia or myeloproliferative disorder. These immunophenotypic findings indicate a heterogeneity in the level of maturation of the clonogenic population, not only in cases of de-novo AML, but also in AML thought to derive from multipotential stem cells.
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PMID:Immunophenotype of clonogenic cells in myeloid leukaemia. 316 52

PMN elastase is a useful additional parameter in the differential diagnosis of the leukaemias. In all patients with myelocytic leukaemias there were elevated levels of elastase-alpha 1-proteinase inhibitor (E-alpha 1PI), while in the lymphatic leukaemias complexed elastase levels were decreased. The highest values were found in the peripheral blood plasma and bone marrow plasma of patients with CML. Despite high E-alpha 1PI concentrations there were no signs of bleeding or consumption of plasmatic coagulation factors. In AML a wide range of E-alpha 1PI levels was observed, extending from slightly elevated to four hundred-fold increased. In myeloblastic leukaemias without maturation (FAB M 1) the concentrations of complexed elastase remained below 150 ng/ml. In myeloblastic leukaemias with maturation (FAB M2) the E-alpha 1PI values ranged between 214 ng/ml and 850 ng/ml (means = 402 +/- 69), and in myelo-monoblastic leukaemias (FAB M4) between 450 ng/ml and 720 ng/ml (means = 663 +/- 72). The only case of promyelocytic leukaemia (FAB M 3) exhibited an extremely high value of 4,550 ng/ml, while a monocytic leukaemia (FAB M5) showed an extremely low value of 5 ng/ml. During cytostatic therapy there was a rapid decrease in levels of complexed elastase, with E-alpha 1PI values returning to normal in remission. In recidivating cases there was an increase of E-alpha 1PI levels in AML and a decrease in ALL. There was a correlation between the E-alpha 1PI concentrations in peripheral plasma and leukaemic bone marrow infiltration, so providing a good basis for monitoring remission from leukaemia and indicating relapse. It was also interesting to observe an extremely low E-alpha 1PI level (5 ng/ml) in patients with myelodysplasia. Under Decortin/Plenastril therapy the concentration rose to 50 ng/ml. An E-alpha 1PI level of 10 ng per ml was observed in one case of Ranitidine agranulocytosis. Under corticoid therapy the value returned to normal within eight days.
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PMID:The importance of granulocyte elastase in haematological diagnosis. 316 79


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