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

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

A total of 165 patients were entered into this study and 140 were evaluate for effects and 165 for toxicities. Of 39 patients with chronic myelogenous leukemia (CML) 21 achieved complete remission (CR), 6 achieved partial remission (PR) with a response rate of 69.2%. In MDS, of 11 patients with chronic myelomonocytic leukemia (CMMoL), one good partial response and 4 partial response were observed (CR + PR:45.5%); of 14 patients with RAEB, one complete response, 4 partial response (CR + PR: 35.7%); of 11 patients with RAEB in T, 3 partial response were observed (response rate: 27.3%). Of 13 patients with polycythemia vera, 4 excellent effect and 7 moderate effect (84.6%) were observed. Seven of 30 patients with acute myelocytic leukemia achieved partial response (23.3%). Mean dosages of SM-108 until remission were 400-500 mg/m2/day on CMMoL, RAEB in MDS, polycythemia vera and CML, and 600-800 mg/m2/day on RAEB in T and AML. In the analysis of adverse effects of SM-108, a subjective side effects including mainly gastrointestinal toxicities were observed in 38 cases (23.0%) of the patients : 26 patients (15.8%) showed objective side effects including liver dysfunction, but these symptoms were transient and not serious. Our study indicates that SM-108 is useful agent against MDS, especially CMMoL, RAEB, RAEB in T, polycythemia vera and CML.
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PMID:[Phase II study of SM-108 (4-carbamoylimidazolium-5-olate) in hematological malignancies]. 264 92

Criteria for the evaluation of chemotherapy for acute leukemia, chronic leukemia, Myelodysplastic syndrome and polycythemia vera were discussed. In leukemia patients the changes in the number of leukemic and normal cells are easily quantitatively evaluated. The criteria depends on the reduction and recovery of leukemic cells and normal cells. In acute leukemia because considerable parts of complete remissions ended with relapse, the evaluation seems necessarily to differentiate good remission from standard remission. For such purpose 5,000 leukocyte differential seemed effective. In the phase II study of anti-leukemia drugs, however, it seemed necessary to find efficacy less than remission, to avoid underestimation of drug efficacy because pretreated patients are usually studied in the phase II study. In the evaluation of chronic myelogenous leukemia, chronic myelomonocytic leukemia or polycythemia vera, short term judgment needs to be further studied about the correlation with longterm efficacy such as survival. The treatment of myelodysplastic syndrome is very hard to evaluate, reduction of blasts and increase of normal cells may be necessary for the improvement of symptoms. The relation of the efficacy and survival seemed necessary to be studied.
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PMID:[Evaluation of chemotherapy of hematological malignancies]. 264 5

Seventy cases of chronic myelogenous leukemia (CML) were analyzed for the presence of ras mutations using polymerase chain reaction (PCR), oligonucleotide hybridization, and direct PCR sequencing. All cases had preceding cytogenetic and bcr rearrangement studies. Aberrant ras genes were detected in none of 39 patients with Philadelphia (Ph) chromosome or bcr/abl rearrangement positive chronic-phase CML and in only 1 of 18 patients in blast crisis, suggesting that ras mutations have little or no role in initiation or progression of common CML. Seven of 13, or 54% of patients with bcr/abl rearrangement negative chronic phase CML (atypical CML) harbored mutations in ras, however. This high incidence of ras mutations, together with the absence of bcr/abl rearrangement, provides evidence that atypical CML is an entity that is molecularly distinct from common CML. Moreover, the clinical characteristics and the high frequency of ras mutations suggest that atypical CML may constitute a subset of the myelodysplastic syndrome and may be best classified as a variant of chronic myelomonocytic leukemia (CMML).
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PMID:Mutations of the ras protooncogenes in chronic myelogenous leukemia: a high frequency of ras mutations in bcr/abl rearrangement-negative chronic myelogenous leukemia. 268 96

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 tried to treat 13 patients with myelodysplastic syndromes (MDS), leukemias and myeloproliferative disorders, with alfacalcidol for their hematological improvement. Eight of them had MDS, 2 acute leukemia (M3, M4), 1 chronic myelogenous leukemia and 2 primary myelofibrosis. All patients were untreated except for 3 patients (PASA, RAEB, AML-M4) who had been treated with mepitiostane, prednisolone and BH.AC-AMP regimen, respectively, prior to alfacalcidol therapy. All patients received alfacalcidol orally for at least one month. The dosage of alfacalcidol ranged from 0.25 to 10 micrograms/day, and the medicine was administrated intermittently when the dosage exceeded 6 micrograms/day to prevent hypercalcemia. The therapeutic effectiveness of alfacalcidol was evaluated according to a criteria by Koeffler (Cancer Treat Rep 69: 1399, 1985) with minor modifications. Three patients (PASA, RAEB, CMML) showed partial response, 3 (RAEB, RAEB in T, AML-M4) minor response and rest of the patients did not respond. The hematological improvement of 6 responders was transient (from 1 to 2 months), however, one patient (PASA) is still responding to alfacalcidol therapy (0.25 microgram/day) for over 12 months. The dysplastic features of hemopoietic cells in the bone marrow showed no noticeable change during the hematological improvement in these responders, suggesting the improvement was obtained as a result of alteration in the proliferation or differentiation of neoplastic clone. None of 13 patients developed hypercalcemia. One patient (AML-M4) became excitable on high dose alfacalcidol (10 micrograms/day). In conclusion, alfacalcidol therapy is effective in some patients with MDS or leukemias and appears worthy especially in the clinical state in which chemotherapy is not indicated.
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PMID:[Therapeutic effectiveness of vitamin D3 in patients with myelodysplastic syndromes, leukemias and myeloproliferative disorders]. 271 94

In vitro megakaryocytic colony formation by progenitors from the bone marrow was studied in 40 myelodysplastic syndrome patients. Megakaryocytic colonies were decreased in number or absent in 30 patients; only 10 showed normal colony growth. The growth correlated to some extent with the FAB-class. Patients with normal colony formation had either RA or RARS, but also in these two FAB-types half of the patients showed reduced megakaryocytic colony formation. Only 1 out of 15 patients with RAEB or RAEBt had normal megakaryocyte growth. The patients with CMML did not show any megakaryocytic colonies. The growth of erythroid colonies was normal in 3 patients and reduced or absent in the others. All 3 with normal erythroid colony formation also showed normal megakaryocyte growth, and all patients with normal megakaryocyte colony formation also had normal granulocyte-macrophage colony growth. Granulocyte-macrophage colony and cluster formation was normal in 17 patients. Defective formation of megakaryocytic, erythroid, and granulocyte-macrophage colonies was seen in 22 patients, compatible with a defect in a pluripotent stem cell. Megakaryocytic colony formation had no obvious correlation with any specific chromosome abnormality, and the distribution of the growth patterns was almost similar in patients with a normal and those with an abnormal karyotype.
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PMID:Colony formation by megakaryocyte progenitors in myelodysplatic syndromes. 227 Oct 49

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

We have examined the efficacy of various drugs in 44 patients with MDS and found the different effectiveness which depends on the type of MDS. Namely, RA appears to respond to steroid hormone, androgen, and/or vitamin D3, regardless of single or combined use. In particular, it is obvious in androgen, and as our previous reports, high content of acidic ferritin in RBC with RA have changed to more basic ones by treatment with androgen. On the contrary, these drugs were not effective on RAEB, RAEB-T, and CMML. A long-term observation is needed to determine whether the prolonged or decreased occurrence of leukemia could be obtained in the effective cases with RA. Most of the cases who did not develop overt leukemia during this study died of bleeding or infections due to thrombocytopenia or leukocytopenia, thus indicating that supportive therapies are important in patients with MDS. Since it has recently been reported that recombinant G-CSF or GM-CSF is helpful to increase the number of leucocyte and to enhance their functional recovery in MDS, these factors may be powerful agents against infections when they are carefully used with regard to the activation of leukemic clones.
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PMID:[Therapy of the preleukemic state: effect of androgens on refractory anemia]. 283 1

Acute myelocytic leukemias (AML) are characterized by a remarkably high incidence (approximately 30%) of point mutations affecting codons 12, 13, or 61 within ras genes. A predominant involvement of N-ras sequences has been established. Neither Philadelphia chromosome-positive chronic myelocytic leukemia nor other chronic myeloproliferative disorders show a similar frequency. However, a proportion of myelodysplastic syndromes, namely, the chronic myelomonocytic subtype (CMML) also show this molecular feature. The following is a brief discussion of the possible biologic and clinical implications of these observations.
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PMID:Mutations in ras genes in myelocytic leukemias and myelodysplastic syndromes. 306 84


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