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

Twenty three Zimbabwean African patients who satisfied the French-American-British(FAB) diagnostic criteria for the myelodysplastic syndromes(MDS) at Godfrey Huggins School of Medicine, University of Zimbabwe, between July 1985 and June 1987 are presented. The disorders appear not to behave differently from those reported in Caucasian populations with regard to clinical and haematological features. Refractory anaemia (RA) occurred in 12 (52.2%) patients; refractory anaemia with ringed sideroblasts (RARS) in 4 (17.4%) patients; refractory anaemia with excess blasts (RAEB) in 2 (8.7%) patients; refractory anaemia with excess blasts in transformation (RAEB-T) in 3 (13.0%) patients; while chronic myelomonocytic leukaemia (CMML) was observed in 2 (8.7%) patients. In 19 cases, the disease was primary and in 4 prior exposure to myelotoxic agents resulted in secondary MDS. The clinical significance of recognising the disorders is briefly high-lighted together with our current treatment protocol.
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PMID:Myelodysplastic syndromes (MDS) in Zimbabweans--preliminary observations. 259 23

Clinical features, prognosis and factors influencing survival, as well as long-term evolution of the disease were analyzed in 130 patients with myelodysplastic syndromes (MDS) with particular reference to the refractory anemia with excess of blasts (RAEB). Survival of patients with 3 FAB subtypes, RAEB, RAEB in transformation (RAEB-T) and chronic myelomonocytic leukemia (CMMoL) showing excess blasts was uniformly poor relative to primary acquired refractory anemia (PARA) and primary acquired sideroblastic anemia (PASA). The degree of cytopenias, karyotypic abnormalities, bone marrow cellularity, transition to acute leukemia were not reliable prognostic parameters for discrimination of RAEB with poor or good prognosis. Disease transition was frequently observed in our MDS patient population, at an overall incidence of 37.7%. Transition of PARA to RAEB occurred after a prolonged course in some patients.
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PMID:Clinical aspects of the myelodysplastic syndromes (MDS) with special reference to refractory anemia with excess of blasts (RAEB). 261 56

Myelodysplastic syndromes (MDS) are disorders characterized by a profound impairment of proliferation and maturation of hematopoietic cells. The prognosis is poor owing to the occurrence of severe cytopenia or to the common leukemic transformation of these conditions. At present there is no available effective treatment for patients with MDS. A patient is reported with simple refractory anemia which responded to therapy with high doses of maturative factors (folic acid and vitamin B12). This therapy resulted in the disappearance of transfusion requirements and in the increase of peripheral blood cell counts. It is concluded that, in view of the lack of toxicity of the treatment with madurative factors, this therapy should be attempted in a sequential fashion in all patients with MSD.
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PMID:[Simple refractory anemia. Response to treatment using maturative factors]. 262 53

A patient with M2-ANLL and a 46,XX,del(5)(q22q33), t(2;11)(p21;q24) karyotype is described. The diagnosis was made after a short period of myelodysplastic syndrome. After chemotherapy consisting of Daunorubicin and Arabinosylcytosine in continuous infusion, the patient reached a complete remission. The chromosome pattern described here has been observed in two other patients with refractory anemia and refractory anemia with excess of blasts, respectively. The breakpoints on the chromosomes 2, 5 and 11 allow us to hypothesize the involvement of N-myc, c-fms, GM-CSF and IL-3 genes.
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PMID:5q- and t(2;11) in a patient with M2 acute non-lymphocytic leukemia. Case report. 262 43

Several aspects of prognosis of myelodysplastic syndromes were reviewed with special attention to refractory anemia (RA). The median survivals were 14 months in chronic myelomonocytic leukemia, 16 months in RAEB, 42 months in RA, and 58 months in RA with ring sideroblasts (RARS). Cumulative leukemia-free rates at 5 years were 31% in RAEB, 80% in RA, and 92% in RARS. The proportion of cases having very low hazards for leukemic transformation or for nonleukemic death was 92% (RARS), 73% (RA), and 26% (RAEB) for leukemic transformation and 23% (RA) and 29% (RAEB) for nonleukemic death. All RARS cases had hazard for nonleukemic death. In RA, the annual mortality rate was about 5 to 11 times higher than that of age-and sex- matched general population up to 6 years. After which no failure was found in RA cases with survival rate of 33% up to 14 years. The relative importance of hazard from leukemic transformation to nonleukemic death in RA was about one half at presentation, but this declined to less than 10% after 10 years.
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PMID:Prognosis of refractory anemias. 262 62

Certain hematopoietic disorders and immunodeficiency states are known to carry a risk of developing acute nonlymphocytic leukemia. In the past some of them have been classified by a variety of terms ranging from refractory anemia to preleukemia, but are currently grouped into a new concept of the myelodysplastic syndromes (MDS). The purpose of this article is to briefly review the updated knowledge of the MDS with emphasis on the clonal origin, natural history and mechanisms of leukemogenesis.
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PMID:[Preleukemic disorders]. 264 30

A 60-year-old patient with a myelodysplastic syndrome (MDS) corresponding to refractory anemia with an increase in blast cells (RAEB) was treated with granulocyte-macrophage colony stimulating factor (GM-CSF) and erythropoietin (EPO) for severe symptomatic pancytopenia. During the GM-CSF treatment a distinct increase in granulocytes was observed, but the reticulocytes and thrombocytes decreased to the point where treatment had to be discontinued after eight days. After subsequent treatment with EPO the reticulocyte count rose from 0% to 2%. However, this rise alone was insufficient to decrease the number of blood transfusions required. The thrombocyte count rose to the original values after the cessation of GM-CSF therapy while continuing treatment with EPO. Bone marrow investigations were performed before and after GM-CSF treatment and indicated a distinct increase in the myeloid precursor cells after therapy, without an increase in blasts. On the other hand, an obvious decrease in erythro- and megakaryopoiesis was observed.
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PMID:[Combined GM-CSF and erythropoietin therapy in myelodysplastic syndrome]. 269 67

The wide prognostic variability of myelodysplastic syndromes (MDS) complicates decision-making regarding the choice and evaluation of alternative treatments to transfusional and antiinfectious supportive measures. Due to its simplicity the Bournemouth scoring system seems to have achieved wide acceptance for establishing the prognosis in MDS patients. The aims of this study were to examine the Bournemouth system in a series of 370 patients with MDS and to evaluate the capability of the prognostic index recently proposed by our group to better define the outcome predicted by the former. The Bournemouth scoring system identified 3 risk groups, A (0-1 points), B (2-3 points) and C (4 points), in the whole series (p less than 0.0001) and it allowed us to stratify refractory anemia (RA), RA with excess of blasts (RAEB) and RAEB in transformation (RAEB-t) patients into two distinct prognostic groups (p = 0.03 and p = 0.01, respectively). This scoring system did not show a significant value in RA with ringed sideroblasts (RARS) and chronic myelomonocytic leukemia (CMML) patients (p greater than 0.05). Our prognostic index clearly segregated patients in the whole series into low- (0-1 points), intermediate- (2-3 points) and high-risk (4-5 points) groups (p less than 0.00001) as well as stratifying ARSA (p = 0.0005), CMML (p less than 0.0001) and RAEB and RAEB-t patients (p less than 0.00001) into different prognostic subset, although it failed to demonstrate a significant predictive value in RA patients.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Prediction of survival in myelodysplastic syndrome. Analysis of 2 scoring systems with prognostic value]. 271 Dec 84

We report an autopsy case of myelodysplastic syndrome (MDS) in a 35-year-old male, who presented with pancytopenia and bleedings. Bone marrow specimens disclosed myelofibrosis and hypercellular marrow with more than 60% atypical erythroblasts in the bone marrow cells. Type I or type II blasts were less than 10% of the peripheral blood and bone marrow cells during the clinical course. At autopsy, infiltration by myeloid and erythroid cells and megakaryocytes was noted in the liver, spleen and lymph nodes. According to the FAB classification, this case might be classified into refractory anemia with excess of blasts (RAEB) or RAEB in transformation. However, the remarkable neoplastic proliferation of three haematopoietic cell lines also indicates acute myeloproliferative disorder such as acute myelofibrosis or acute panmyelosis.
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PMID:An autopsy case of myelodysplastic syndrome (MDS): diagnostic problems between MDS and the other haematopoietic disorders including acute myelofibrosis. 274 51

In a series of 121 consecutive patients with a myelodysplastic syndrome (MDS), studied in two laboratories, of which 87 (71.9%) had abnormal karyotypes, twelve had a structural abnormality of the long arm of chromosome 11 (13.8%). There were six deletions, one ring chromosome and five reciprocal translocations, all involving a chromosome band 11q23. Of these twelve patients, five had a refractory anemia (RA) and seven a refractory anemia with excess of blasts (RAEB). RA was associated more frequently with 11q deletions as the sole abnormality, while translocations or multiple chromosome abnormalities were commonly associated with RAEB. The study shows that the 11q aberrations represent frequent structural chromosome rearrangements in MDS.
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PMID:Chromosomes in myelodysplastic syndrome: structural abnormalities of chromosome 11. Czechoslovak MDS Cooperative Group. 275 Dec 48


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