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At least 199 records · Page 11Linked to original sources
PubMed1988

[Effect of low-dose Ara-C regimen on myelodysplastic syndrome (MDS)].

A low-dose Ara C (LDAC) regimen (0.2 mg/kg/d) was tried in two groups of eleven patients with MDS and in ten with hypoplastic leukemia (HL) proven by biopsy. In HL, seven patients achieved complete remission (CR), but CR duration was rather short (4-9 months). Some cases showing relapse could be induced back into CR by the LDAC regimen. All patients showed marked bone marrow aplasia before reaching CR. Therefore, the main effect of LDAC was considered to be cytotoxic activity. In one HL patient, the karyotype of 47, XX, +8 at diagnosis was converted to normal, 46, XX after CR. In MDS, there were no CR cases but five patients partially responded to the LDAC regimen. One patient died of cerebral hemorrhage soon after LDAC treatment. Three patients developed overt leukemia and in one of them the disease was well controlled by LDAC over thirty months. One patient with typical refractory anemia with excess blasts (RAEB) suffered severe bone marrow aplasia after 12 mg/d for 10 days of LDAC, but one month later his hematopoiesis gradually recovered and reached a normal hemoglobin level (12 g/dl). However, all of his karotypes showed 45, XY, -7 at that time. LDAC regimen is effective, especially for HL, but may have a limited effect on MDS.

Aged↗
PubMed1984

Granulocyte Fc-IgG and C3b receptor expression in the primary myelodysplastic syndromes (MDS): relationship with dysgranulopoiesis and evidence for heterogeneity of morphological subgroups.

The expression of Fc-IgG and C3b membrane receptors by granulocytes and their precursors was examined in 78 patients with primary myeloid dysplasia (MDS). The marrows were categorized into five morphological groups, broadly corresponding to those described by the FAB group, and further graded into mild and severe according to the severity of dysgranulopoiesis. Fractionated bone marrow and peripheral blood granulocyte receptors were assessed by rosette formation with optimally sensitized ox erythrocytes and the results compared with those found in 17 normal marrows and with those previously reported in megaloblastic anaemia. Fc-IgG and C3b receptor expression was generally increased in maturing granulocytes in all MDS diagnostic groups but premature expression was particularly marked in chronic myelomonocytic leukaemia and refractory cytopenia. Receptor patterns showed a better correlation with the severity of dysgranulopoiesis than with the morphological types. It is suggested that, as in megaloblastic anaemia, changes in membrane receptor expression reflect nuclear-cytoplasmic asynchrony. The results further indicate considerable immunological heterogeneity of granulocytes within individual MDS categories.

Granulocytes↗
PubMed1995

[Therapy-related leukemia with t(8;21) initially diagnosed as MDS (RAEB in T)].

A 64-year-old woman was diagnosed as having myelodysplastic syndrome (MDS) at 45 months after receiving radiotherapy for advanced carcinoma of the uterine cervix. We chose low dose therapy of SPAC and ACR because of the diagnosis as therapy-related MDS and the existence of radiation colitis. She obtained minor response, but two months later she transformed to AML (M2). The interval between low dose therapies was getting shorter and shorter, so we tried intensive chemotherapy consisting of BHAC, ACR and 6MP. Blast numbers were reduced, but she died of sepsis and intestinal bleeding. The patients of MDS with t(8;21) and the patients of therapy-related AML (tAML) with t(8;21) are very rare. According to the literature, only karyotype is a prognostic factor in AML/MDS with t(8;21). And diagnosis by the criteria of FAB classification is of little value regarding clinical progress. That is to say, if the patient has only t(8;21) or karyotypic abnormalities which are of little value in prognosis, such as the loss of a sex chromosome, it must be treated as de novo AML, but if patient has karyotypic abnormalities such as -5, 5q-, -7, 7q-, and/or multiple (complicated) abnormalities, we must accept that the prognosis is poor and must treat it as ordinary MDS/tAML.

Anemia, Refractory, with Excess of Blasts↗
PubMed1995

[Successful treatment of acute myelomonocytic leukemia developed from MDS with cytarabine ocfosfate (SPAC)].

A 65-year-old female with acute myelomonocytic leukemia (AMMoL) developed from myelodysplastic syndrome (MDS), successfully treated with cytarabine ocfosfate (SPAC) is reported. Ubenimex, calcitriol and corticosteroid had a minor effect on her MDS. Since she had severe anemia and congestive heart failure on developing leukemia, she was treated with oral administration of SPAC, a cytidine deaminase resistant derivative of Ara-C. After the second course of SPAC (200 mg/day, for 14-28 days), marked erythroid bursts were found and she entered complete remission. The samplings of SPAC and its metabolites of SPAC were investigated in 2 cases including this case, but there seemed to be no relation between their content and effects. In AML patients, especially in cases developed from MDS, SPAC might be useful because it can be given orally even in an outpatient.

Aged↗
PubMed1994

Current use of recombinant human erythropoietin (r-huEPO) in the management of symptomatic anaemia in patients with myelodysplastic syndromes (MDS)

PURPOSE: To evaluate the efficacy of subcutaneous erythropoietin (r-huEPO) in patients with myelodysplastic syndrome (MDS) requiring periodical blood transfusion. PATIENTS AND METHODS: The study was performed on 9 MDS patients distributed as follows: 4 with refractory anaemia, 2 with refractory anaemia with ringed sideroblasts, 2 with chronic myelomonocytic leukaemia and 1 with refractory anaemia with excess blasts in transformation. All the patients had been diagnosed at least three months earlier and had a mean monthly transfusion requirement of 3.5 blood units (range, 2-5.3). Baseline serum EPO levels were assessed by ELISA, subcutaneous r-huEPO was given at a doses of 10,000 IU three times a week. A positive response was defined as increase of haemoglobin rates in 1 g/dL or decrease of transfusion requirements in 50%. A negative response was registered when the haemoglobin rates or transfusion requirements were maintained in the patient. The results were evaluated 4,6 and 12 weeks after the beginning of the treatment. RESULTS: Response to treatment was seen in five patients (56%), who showed increased haemoglobin rates and in four of them (45%) no transfusions were needed since the onset of this therapy. The positive response to r-huEPO appeared within the 4th week of treatment. The four unresponsive patients (44%) showed no changes in spite of prolonged treatment. Positive responses correlated with increased red-cell progenitors in the bone marrow and decreased myelo/erythroid ratio. No severe untoward effects were seen with this treatment, and mild local pain at the sites of injection was alleviated by warming the r-huEPO solution before administration. CONCLUSIONS: MDS with symptomatic anaemia can be initially treated with r-huEPO for 4 weeks, although it seems advisable to maintain this treatment in respondents until the maximal response has been attained. On the contrary, when no response is seen in that period treatment must be discontinued.

Aged↗
PubMed1994

Myelodysplastic syndromes (MDS) in Central Africans.

Forty two patients who were seen and satisfied the French-American-British (FAB) diagnostic criteria for myelodysplastic syndromes (MDS) over a 6-year period at the University of Zimbabwe's Department of Haematology, Harare, are presented. Their overall ages ranged from 29 to 75 years with a mean +/- SD of 57.8 +/- 11.2 years. Males outnumbered females with a male to female ratio of 1.2:1. Refractory anaemia (RA) occurred in 33.3%; refractory anaemia with ringed sideroblasts (RARS) in 16.7%; refractory anaemia with excess blasts (RAEB) in 21.4%; refractory anaemia with excess blasts in transformation (RAEB-T) in 16.7% and chronic myelomonocytic leukaemia (CMML) in 11.9% of the patients. In 90.5% the disease was primary and in 9.5% prior exposure to myelotoxic agents resulted in secondary MDS. The study reveals that MDS as a cause of anaemia in the African population is usually hidden in the big number of well known anaemias due to rampant malaria, malnutrition and a host of nutritional deficiencies. There is therefore the need to increase diagnostic awareness among our clinicians about the existence of these disorders.

Adult↗
PubMed1993

[Hematological study in 27 patients with myelodysplastic syndrome (MDS)].

The clinical, histologic, and cytogenetic features of myelodysplastic syndrome (MDS) were analyzed in 27 patients consisting of 21 males and 6 females (M: F ratio = 3.5:1) whose median age was 60.9 years. Analysis of the peripheral blood of the patients revealed pancytopenia (8 patients), bicytopenia (12), and cytopenia in one cell lineage (5). The patients had high serum iron value and low UIBC value. More than 20% of all erythroid cells in the bone marrow consisted of ringed sideroblasts in not only RARS patients but also RAEB, CMMoL, and RAEB-T patients. Morphological changes of the bone marrow cells were observed in patients with all types of MDS. Eighty percent and 60% of RAEB and RAEB-T patients, respectively, showed trilineage myelodysplasia. Chromosomal abnormalities were observed in 52% of MDS patients. The rate of leukemic transformation was high in RAEB, CMMoL, and RAEB-T patients.

Adult↗
PubMed1998

[A combination chemotherapy with cytarabine ocfosfate, low dose etoposide, and G-CSF for the treatment of high risk MDS (RAEB, RAEBt)--a pilot study].

We evaluated a combination chemotherapy with cytarabine ocfosfate (SPAC), low dose etoposide and G-CSF for the treatment of high-risk MDS (RAEB, RAEBt). Seven patients with high-risk MDS were treated with a daily combination, 200 mg/day SPAC p.o., 50 mg/day etoposide p.o. and 75 micrograms/day G-CSF s.c. One patient achieved complete response, 2 achieved good response and one patient minor response. Although all of the patients developed severe marrow hypoplasia after chemotherapy, the nonhematologic adverse effects were mild enough to be tolerated. This combination chemotherapy should be useful in the clinical management of patients with high-risk MDS.

Aged↗
PubMed2004

Hematopoietic stem cell transplantation (HSCT) in children with juvenile myelomonocytic leukemia (JMML): results of the EWOG-MDS/EBMT trial.

Allogeneic hematopoietic stem cell transplantation (HSCT) is the only proven curative therapy for juvenile myelomonocytic leukemia (JMML). We, the European Working Group on Childhood MDS (EWOG-MDS) and the European Blood and Marrow Transplantation (EBMT) Group, report the outcome of 100 children (67 boys and 33 girls) with JMML given unmanipulated HSCT after a preparative regimen including busulfan, cyclophosphamide, and melphalan. Forty-eight and 52 children received transplants from an HLA-identical relative or an unrelated donor (UD), respectively. The source of hematopoietic stem cells was bone marrow, peripheral blood, and cord blood in 79, 14, and 7 children, respectively. Splenectomy had been performed before HSCT in 24 children. The 5-year cumulative incidence of transplantation-related mortality and leukemia recurrence was 13% and 35%, respectively. Age older than 4 years predicted an increased risk of disease recurrence. The 5-year probability of event-free survival for children given HSCT from either a relative or a UD was 55% and 49%, respectively (P = NS), with median observation time of patients alive being 40 months (range, 6 to 144). In multivariate analysis, age older than 4 years and female sex predicted poorer outcome. Results of this study compare favorably with previously published reports. Disease recurrence remains the major cause of treatment failure. Outcome of UD-HSCT recipients is comparable to that of children receiving transplants from an HLA-identical sibling.

Adolescent↗
PubMed1999

The impact of the ART approach on the treatment profile in a mobile dental system (MDS) in South Africa.

The changing profile of oral care rendered through the Mobile Dental System (MDS), after the introduction of the Atraumatic Restorative Treatment (ART) approach is described. During the first year of introduction of ART, the percentages of amalgam restorations and tooth extractions decreased significantly (P < 0.0001). This is partly ascribed to a change in choice of treatment by dental operators in favour of ART and also due to an increase in acceptance by patients because of the reduced fear, and the patient-friendly nature of the ART approach. The reduction in amalgam restorations was 16.0 per cent for permanent and 1.4 per cent for primary posterior teeth. Extraction of posterior teeth was reduced by 17.4 per cent in the permanent and 35.7 per cent in the primary dentitions. The restorative component of oral care increased by 33.4 per cent in the permanent and 37.1 per cent in primary posterior teeth. The one-year survival of one-surface ART restorations using Fuji IX and KetacMolar was 93.6 per cent. Full and partial (more than 90 per cent) retention of the sealant part of the ART restoration was obtained in 75 per cent of the cases after one-year. During the one-year period, infection control was made more simple and this facilitated easier maintenance of mobile dental equipment. The introduction of the ART approach reduced extraction, restored more teeth and made oral care in the MDS more preventive, less threatening and thus more patient-friendly.

Child↗
PubMed1991

Ineffective hemopoiesis in the myelodysplastic syndromes (MDS) as studied by daily in situ observation of colony-cluster formation.

Daily in situ observation of individual proliferating cells was performed to examine ineffective hemopoiesis in vitro. Bone marrow mononuclear cells (BMMNC) from 24 myelodysplastic syndrome (MDS) patients and 12 controls were cultured for granulocyte-macrophage progenitor (CFU-gm) assays using methylcellulose. Individual proliferating cells were mapped at 3 days of culturing and their fates were followed by daily in situ cell counting contained within each cell aggregate until day 8. By retrospective analysis of the daily growth of the cells, a significantly greater proportion of noncolony-forming cells in MDS were found to proliferate initially, but failed to do so thereafter and degenerated in the culture. Cells showing these abnormal growth characteristics apparently contributed to ineffective granulopoiesis. The present method may be useful for clarifying ineffective granulopoiesis.

Adolescent↗
PubMed2001

Reduced CD43 expression on the neutrophils of MDS patients correlates with an activated phenotype of these cells.

CD43 (also known as leukosialin and sialophorin) is a surface sialoglycoprotein expressed at high levels on most leukocytes implicated in adhesion, antiadhesion, and activation/proliferation mechanisms. We studied the expression of this molecule on the leukocytes of patients with myelodysplastic syndromes (MDSs) in an effort to detect acquired deficiencies of this molecule. We used immunofluorescence flow cytometry in analyzing whole blood and isolated neutrophils from 49 MDS patients, 33 men and 16 women aged 33 to 85 years (median, 75 years), and 18 healthy individuals aged 35 to 80 years (median, 72 years). According to French-American-British classification criteria, 13 patients had refractory anemia, 18 had refractory anemia with ringed sideroblasts, 9 had refractory anemia with excess of blasts, 4 had refractory anemia with excess of blasts in transformation to acute leukemia, and 5 had chronic myelomonocytic leukemia. We found decreased expression of CD43 on the neutrophils of these patients, and we correlated this finding with the activation status of these cells as it is defined by their phenotypes. We studied the expression of CD11b, CD18, CD35, CD67, CD69, CD44, and CD53 molecules known to be changed in the activated form of neutrophils. CD43 expression correlated positively with CD53 and CD44 expression and negatively with CD11b, CD18, CD35, CD67, and CD69 expression. Additionally, increased levels of soluble vascular cell adhesion molecules were detected in these patients, suggesting endothelial cell activation. In conclusion, we believe that the decreased expression of CD43 on the neutrophils of MDS patients is associated with activation of these cells and is probably due to cleavage of the molecule from the cell surface and that the same mechanism is possibly responsible for the parallel down-regulation of CD44 and CD53.

Adult↗
PubMed2005

An epigenetic approach to the treatment of advanced MDS; the experience with the DNA demethylating agent 5-aza-2'-deoxycytidine (decitabine) in 177 patients.

During the last 10 years, three European phase II studies were performed to investigate the treatment of elderly patients with myelodysplastic syndrome (MDS) with low-dose 5-aza-2'-deoxycytidine (decitabine, DAC). All these European trial data were reviewed on the basis of the International Prognostic Scoring System (IPSS) risk criteria and the response criteria as recently published by an international working group. To investigate the results in a larger cohort of patients and to determine risk factors, all data were pooled with some observations from the PCH 95-06 US phase II study. The response rate in the 177 patients evaluated (median age 70 years) was 49%. The median response duration was 36 weeks, and the median survival was 15 months. Analysis of the data according to sex, age, French-American-British classification, percentage of blasts in the bone marrow, IPSS risk group, lactate dehydrogenase and cytogenetics did not reveal any factor predictive of response. Overall, 69% of patients benefited, including those with stable disease during therapy. Response duration was significantly shorter with increasing risk (according to the IPSS classification). Haemoglobin level and neutrophil count showed an inverse correlation to the IPSS classification. Univariate analysis showed a significantly inferior survival for elderly patients (>75 years of age) and for those with high levels of serum lactate dehydrogenase (LDH) (more than two times the normal values). Patients with high-risk cytogenetic abnormalities according to the IPSS risk criteria showed better overall survival than those with intermediate-risk abnormalities. When analysed according to the IPSS risk classification, high-risk patients had worse survival prospects following decitabine therapy than those with intermediate risk; however, compared to the originally reported IPPS outcomes for high-risk patients, they probably showed better survival. During the treatment period, 18% of the patients progressed towards acute leukaemia. Decitabine showed a rather low toxicity profile in this elderly patient group. In conclusion, low-dose decitabine is an active drug for the treatment of MDS patients, even for those older than 75 years with bad prognostic characteristics.

Adult↗
PubMed1999

Myelodysplastic syndrome (MDS) associated with increased hemoglobin F and trisomy 8: presentation of a patient.

A 7.5 year old boy with myelodysplastic syndrome (MDS) of CMML type associated with trisomy 8 and elevated hemoglobin F (Hb F) value is presented. Hematological evaluation of the patient revealed that the Hb was 10 g/dl, MCV 110 FL, platelets 58 X 10(9)/l, WBC 5.4 X 10(9)/l with 24% atypical monocytes. Karyotype analysis revealed 47, XY, +8. Hb F value was 21% which was distributed heterogeneously among red cells. PCR amplified cDNA copies of circulating reticulocyte mRNA were used to measure the relative amounts of alpha-, beta-, and gamma- globin. There was marked increases in both alpha/beta mRNA ratio (20%) and gamma/(gamma+beta) mRNA ratio (35%) in the patient compared to normal subjects. The study indicated that increased transcription of alpha and gamma genes are partly responsible for the elevation of Hb F in MDS.

Base Sequence↗
PubMed1992

Myelodysplastic syndromes (MDS) and acute myeloid leukemias (AML) with myelofibrosis.

Acute myeloid leukemias (AML) and myelodysplastic syndromes (MDS) enter rarely in the differential diagnosis of myelofibrosis (MF). MF of marked intensity, resulting in either "dry taps" or non-representative smears, is encountered in approximately 10% of cases. MF may be observed in any type of AML, most frequently in acute megakaryoblastic leukemia (M7). Apart from some typical cases of MDS, MF is associated with cases of acute myelodysplasia with myelofibrosis (and a major megakaryocytic component). This syndrome has been described under various headings: acute or malignant myelosclerosis, and acute MF. It should be distinguished from M7 and from myeloproliferative syndromes.

Acute Disease↗
PubMed1992

Clinical features of MDS.

MDS is primarily a disease of the elderly. Cases who give a history of exposure to X-rays, cytotoxic drugs or leukaemogenic chemicals may be younger. Many cases of MDS present because of an incidental blood count. The most prominent clinical features are those of anaemia, neutropenia, thrombocytopenia. Because haemopoietic tissue is also dysfunctional the pathological effect is often greater than the figures would suggest, even leading to infection of bleeding with normal neutrophil or platelet counts. Occult abscesses are a particular feature. Despite documented abnormalities of the lymphoid system, neither infections characteristic of T-cell immunodeficiency nor autoimmunity is a problem. The proliferation of monocytes in CMML leads to organomegaly, leukaemia cutis, serous effusions and vasculitic lesions caused by the mishandling of circulating immune complexes. Cancer is no commoner than in age-matched controls, but coincident lymphoid tumours do occur. Many patients require long-term blood transfusion and will run into problems of iron overload unless precautions are taken.

Anemia↗
PubMed1995

Karyotype at diagnosis, subsequent leukemic transformation and survival in myelodysplastic syndrome. Czechoslovak MDS Cooperative Group.

240 patients with MDS studied cytogenetically at diagnosis between 1981 and 1990 were followed until death or until April 1992 to evaluate the prognostic significance of FAB classification, age and karyotype. 61 patients (25.4%) subsequently transformed into AML and 176 (73.3%) died during the follow-up period. Patients with blastic MDS types had a shorter survival and a higher probability of leukemic transformation. The younger age increased the probability of leukemic transformation, but was associated with a longer survival. The absence of analyzable mitoses was associated with a shorter survival. The complex chromosomal abnormalities at the initial evaluation identified a subgroup of patients with a high risk of a short survival and/or subsequent leukemia transformation. In refractory anemia the presence of complex chromosomal abnormalities was linked with a relative risk of 3.58 of leukemic transformation and shorter survival as compared with other cytogenetically defined groups.

Age Factors↗
PubMed1996

Immunophenotyping of AML and MDS and detection of residual disease.

Immunophenotyping improves both accuracy and reproducibility of the FAB classification and is considered particularly useful for identifying poorly differentiated FAB subtypes of AML, such as AML with minimal differentiation (M0), microgranular promyelocytic leukaemia (M3V), and megakaryoblastic leukaemia (M7). Immunological studies of myeloid leukaemic blasts has become critical also in identifying biphenotypic leukaemias and AML expressing lymphoid-associated markers (Ly+ AML). At present, while the prognostic value of individual antigen expressions is still controversial, due to technical questions, the immunological detection of MRD seems to be important in monitoring AML patients in remission and, perhaps, in detecting leukaemic cell contamination into bone marrow or peripheral blood progenitor cells collected for autologous transplantation. In addition, the relationship established between genetic abnormalities and certain phenotypes within different FAB subtypes suggests that, in the future, immunophenotypical studies could be used for the screening of AML cases carrying specific genetic aberrations. Compared to acute leukaemias, little information is available concerning immunological patterns in MDS, and the role of the immunophenotype in diagnosis, subclassification, and prognosis of MDS is currently not well established.

Acute Disease↗