Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We retrospectively reviewed 62 MDS patients (15 RA, 3 RARS, 10 CMML, 20 RAEB, 14 RAEBT) to clarify the current problems in their management. Median survival of RA and RARS patients was 67.9 months and significantly longer than that of CMML, RAEB, or RAEB-T patients with median survivals of 16.1, 16.8, and 9.5 months, respectively. Karyotypic abnormalities were observed in 58% of the patients examined. Forty-two patients died, 16 (38%) of leukemic transformation and 21(50%) of bone marrow failure. While most of the RAEB-T patients of all ages and all the RAEB patients diagnosed below 60 years of age died of transformation, 70% of the older RAEB patients died of infection. Prognosis after transformation was poor and 12 patients died within two months. These results indicate that management after transformation and treatment against infection in RAEB patients with advanced age are crucial to improve the prognosis in MDS.
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PMID:Prognostic implications in myelodysplastic syndromes: A review of 62 cases. 917 14

We report on a patient with Fanconi's anemia (FA) who developed a myelodysplastic syndrome (RAEB-T) with complex karyotypic abnormalities (trp 1q23q42, monosomy 20, trisomy 13) at the age of 28. The patient achieved a complete hematological and cytogenetic remission after treatment with sequential high-dose cytosine arabinoside/mitoxantrone followed by G-CSF (5 micrograms/kg). Bone marrow hypoplasia was prolonged with 38 days of granulocytopenia < 500/microliters and 62 days of platelet transfusion dependency. Nonhematological toxicity did not exceed that of patients without underlying FA. Remission duration was 7 months. This observation shows the feasibility of high-dose Ara C treatment in patients with FA and MDS. Although hematopoiesis remained clonal in remission, the suppression of the cytogenetically abnormal clones transiently reversed the antecedent long-lasting pancytopenia.
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PMID:Induction of a hematological and cytogenetic remission in a patient with a myelodysplastic syndrome secondary to Fanconi's anemia employing the S-HAM regimen. 923 12

Twenty eight patients of myelodysplastic syndrome (MDS) were treated with low dose cytosine arabinoside to study the effect of this treatment modality. All patients presented with a hemoglobin of less than 12 Gm/dl, 4 (15%) had neutropenia with an absolute neutrophil count of less than 500 x 10(6)/L and 18 (65%) had thrombocytopenia of less than 100 x 10(9)/L. The subtypes according to the bone marrow evaluation included 14 patients of refractory anemia with excess blasts (RAEB), 10 refractory anemia with excess blasts in transformation (RAEB-T), and 4 chronic myelomonocytic leukemia (CMML). Five patients (18%) achieved complete hematological response, 10 (36%) had a partial response and 9 (33%) patients had no response. Four patients died early during treatment due to tumor lysis (1 CMML) and hemorrhage (3 RAEB). Seven patients progressed to acute myeloid leukemia (AML) while on therapy and three progressed to AML after completion of therapy. Five patients died of hemorrhage and 3 of septicaemia after achieving an objective response. The mean duration of follow up in these patient was 8 months (range 1 month-3 years). Only 3 patients of RAEB have survived for greater than 2 years. Our data reveals the short term benefit of this mode of therapy and emphasizes the need to develop newer therapeutic approaches.
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PMID:Low dose cytosine arabinoside in the treatment of myelodysplastic syndrome. 925 14

The percentage of long-term survivors after intensive chemotherapy and the outcome of MDS patients who achieve partial remission (PR) with intensive chemotherapy (IC) are not known. Between 1981 and 1996 we treated 99 patients with de novo MDS who had high-risk MDS or progression to AML, with IC. 41 (41%) achieved CR, 16 (16%) achieved partial remission (PR), 26 (26%) had failure, and 16 (16%) died in aplasia. Eight of the patients who achieved CR were autografted, three were allografted and the remaining cases received moderate consolidation chemotherapy. After IC, the 16 PR patients fulfilled the criteria for RA in 15 cases and CMML in one case. Median PR duration was 17 months, and three PR were > 3 years (39, 50+, 82+ months). Median actuarial survival of patients who achieved PR and CR was 18 months and 20 months from the onset of IC, respectively (difference not significant). Of the 71 patients treated before 1993, with sufficient follow-up, 10 (14%) had survived > 4 years (long-term survivors). Four of them were alive in first CR after 49+ to 110+ months and probably cured, two were alive in PR after 50+ and 82+ months and four had died after 49-78 months. Long-term survivors were characterized by a significantly higher incidence of RAEB-T at diagnosis, and with normal or favourable cytogenetic findings. In patients with RAEB-T at diagnosis included before 1993, 8/23 (35%) cases who had no unfavourable karyotype had survived > 4 years. Our findings suggest that MDS patients who achieve PR with IC, and not only those who achieve CR, can benefit from this type of treatment. The percentage of long-term survivors remains low, however, and is almost restricted to patients with RAEB-T at diagnosis and no unfavourable karyotype.
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PMID:Long-term follow-up of de novo myelodysplastic syndromes treated with intensive chemotherapy: incidence of long-term survivors and outcome of partial responders. 932 99

During 1990 to 1993, five patients who had been treated in our hospital for aplastic anemia (AA) with a normal karyotype, were found to have hypoplastic myelodysplastic syndromes (hMDS); three patients with RA, one RAEB, and one RAEB-T, and all of them have chromosomal abnormalities. The mean age of the hMDS patients (4 men, 1 woman) was 51.0 years (range 31-61). We retrospectively studied the hematological features of the 5 cases in the early stage without chromosomal abnormalities. They showed, 1) appearance of erythroblasts (3/5), 2) more reticulocyte counts than typical AA (mean 7.8 x 10(4)/microliter), 3) macrocytic anemia (mean MCV 106fl) in peripheral blood, 4) relative erythroid hyperplasia (M/E ratio 0.2-1.5), and 5) low lymphocyte counts (mean percentage of lymphocytes 32, 8%) in bone marrow. Considering about the differences or transformation between AA and hMDS, it is interesting that all five patients have initially had a normal karyotype and subsequently developed chromosomal abnormalities.
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PMID:[Hematological characteristics of hypoplastic MDS patients with chromosomal abnormalities, who had been followed for aplastic anemia with normal karyotypes]. 945 42

The demonstration of synergistic interaction between differentiation inducing agents and DNA synthesis inhibitors suggests that these two groups act by two different mechanisms. We prospectively studied the response rate, response duration, survival, and toxicity in 10 patients with myelodysplastic syndrome (MDS) treated with all trans retinoic acid (ATRA) and low dose cytosine arabinoside (ara-C). These patients diagnosed between October 1993 and May 1995 were treated with ATRA (45 mg/M2/day) for 90 days followed by 90 mg/M2 on alternate day till Day 275; together with Ara-C (10 mg/m2) subcutaneously twice daily for 21 days for a total of 6 cycles. These patients were analyzed for response after 3 cycles of LD Ara-C and at the time of completion of therapy. Toxicity was recorded at the end of each cycle of Ara-C. There were 6 male and 4 female patients in the age range of 24 to 76 years. The morphological diagnosis was chronic myelomonocytic leukemia in 2, refractory anemia with excess blasts in 4 and refractory anemia with excess blasts in transformation in 4. Only 1 patient achieved a complete remission and 1 patient achieved a partial response. Four patients had progressive disease on treatment. One patient died of neutropenic sepsis and 1 of resistant thrombocytopenia and intracranial hemorrhage while on treatment. One patient refused further treatment after a minor clinical response and in 1 patient treatment was stopped due to toxicity. This data in a pilot study with a limited number of patient suggests that ATRA in combination with Ara-C has little effect in MDS.
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PMID:All trans retinoic acid with low dose cytosine arabinoside in the treatment of myelodysplastic syndrome. 963 88

Myelodysplasia (MDS) is mostly characterized by a normal or increased number of normoblasts in the bone marrow and an impaired in vitro colony formation. In the present study we analyzed whether this might be due to a disconnection between proliferation and differentiation. CD34+/CD36- sorted bone marrow cells of 18 MDS patients were cultured in a clonogenic and suspension culture assay in the presence of erythropoietin (Epo) and mast cell growth factor (MGF). Burst-forming units erythroid (BFU-E, 75 +/- 88/10(4) CD34+ cells, X +/- s.d.) and colony-forming units E (CFU-E) were observed in eight of the 13 cases (62%) with refractory anemia with or without ring sideroblasts (RA and RARS) and one of the five cases with RA with excess of blasts or in transformation (RAEB and RAEB-T). Suspension cultures with CD34+/CD36- sorted cells with Epo plus MGF demonstrated an 8.9 +/- 6.5-fold expansion after 7 days in cases with >10 BFU-E/10(4) CD34+/CD36- cells while cases with <10 BFU-E/10(4) CD34+/CD36- cells demonstrated 1.0 +/- 0.8-fold expansion especially in cases with RAEB/RAEB-T. FACS and morphology analysis after 7 days of suspension culture demonstrated partial differentiation along the erythroid lineage in cases with RA/RARS (75%) and RAEB/RAEB-T (66%) reflected by the presence of erythroblasts and normoblasts with variable expression of CD34, CD36 and Glycophorin A. In cases with erythroid colony formation 69 +/- 24% of the cells were CD34-/CD36+ and in cases with <10 BFU-E/10(4) CD34+ cells 18 +/- 16% of cells were CD34-/CD36+. Iron staining showed the presence of ring sideroblasts in two cases with RARS indicating that the cells originate from the abnormal erythroid clone. Finally, it was shown that cases with an impaired proliferative response demonstrate an enhanced binding of Annexin-V on CD34+ cells during the first days of the cell suspension culture phase. These results suggest that a defect in the proliferative response is most pronouncedly expressed in MDS whereas a subpopulation of cells retain the capacity to differentiate between transition to a terminated stage.
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PMID:CD34+/CD36- cells from myelodysplasia patients have a limited capacity to proliferate but can differentiate in response to Epo and MGF stimulation. 1008 48

To gain more insight into the understanding of myelodysplastic syndromes (MDS) as they occur in Thailand, a retrospective clinicopathologic analysis was conducted in patients (age > 15 years) diagnosed as MDS from January 1992 to December 1996 at the five major medical centers in various geographic regions of the country. The central reviewers independently examined the bone marrow and peripheral blood smears of all the patients and classify the disease according to the French-British-American (FAB) classification. There were a total of 117 eligible patients. The median age of the patients was 56 years (range 16-86). The male:female ratio was 1:1. Thirty-two percent of the patients were younger than 40 years. The frequency of the FAB subtypes was RA/RARS, 54.7; RAEB, 23.1; CMML, 9.4; and RAEB-T, 12.8%. Anemia was the most common symptom presenting in 84.6% of the patients. In the 34 patients in whom the cytogenetics in the bone marrow were analysed, 44.1% revealed abnormalities. Of these, monosomy 7 and trisomy 8 were the most common aberration, each being detected in 26.7% of the patients. Transfusions were the main therapeutic modality in 80% of the patients. Kaplan-Meier analysis revealed a 5 year survival rate of 29% for the whole group with a median survival of 24 months. Twenty-five percent of the patients had progressed to acute myelogenous leukemia (AML) with a median time to disease-progression of 23 months. The median survival for RA/RARS, RAEB, CMML and RAEB-T were 58.4, 19.9, 10.7 and 8.7 months, respectively (P < 0.001). The stepwise Cox regression analysis revealed the percentage of blasts in the bone marrow as the only parameter significantly associated with survival and disease progression. On comparison with data from other countries, the age of Thai patients with MDS is considerably lower than the western population but is comparable to other asian countries. The distribution of the FAB subtypes and the survival of the patients are similar. The major prognostic features, however, lie in the percentage of blasts in the bone marrow rather than the degree of the observed cytopenia.
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PMID:Myelodysplastic syndromes in Thailand: a retrospective pathologic and clinical analysis of 117 cases. 965 32

Fourty children with MDS treated in seven centres of The Polish Children's Leukemia Lymphoma Study Group in period 1975-1998y were included to the study. In 16 children RAEB-T, in 2 CMML in 10 RA and in 12 RAEB were diagnosed. Our and literature data showed that BMT is the best therapy for children with MDS. For children, who don't have a donor for BMT. Roacutan therapy seems to be the most effective.
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PMID:[Treatment outcome for myelodysplastic syndromes (MDS) obtained by the Polish Children's Leukemia/Lymphoma Study Group]. 968 18

We investigated double (specific and nonspecific) esterase (DE) staining in marrow cells of 237 patients with the myelodysplastic syndromes (MDS). Additional abnormalities of neutrophilic granules were examined cytochemically and immunocytochemically for myeloperoxidase activity and antigen elastase, lactoferrin and CD15 granule-membrane glycoproteins. Abnormal DE staining (>/=3% of all nucleated marrow cells) was present in 27% of patients with no difference among different MDS subtypes. However, the prevalence of high abnormal DE staining (>/=10%) was significantly lower in refractory anemia with excess blasts in transformation (1%) compared to other MDS subtypes (12-15%; p = 0.004). The prevalence of other granule abnormalities was not statistically different in the DE normal and DE abnormal groups. Abnormal DE staining is relatively common among all MDS subtypes. High DE staining may identify a subgroup of patients with a lower grade MDS.
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PMID:Double esterase staining and other neutrophilic granule abnormalities in 237 patients with the myelodysplastic syndrome studied by the cancer and leukemia group B. 969 Nov 40


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