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)

Twenty-three patients with bicytopenia or pancytopenia were retrospectively studied. The patients with underlying disorders, blast count of more than 5% on bone marrow (BM) aspirate, blast count of more than 1% on peripheral blood or ringed sideroblast count of more than 15% on BM aspirate were excluded. According to Yoshida's criteria, 23 patients were classified into 6 subtypes [AA (aplastic anemia)1: typical AA, AA2: atypical AA, MDS (myelodysplastic syndrome)3: typical RA (refractory anemia, MDS4-6: atypical RA], and AA1 7 cases; AA2 2 cases; MDS3 5 cases; MDS4 1 case; MDS5 2 cases; MDS6 6 cases. To clarify the clinical features of atypical RA group (MDS4-6), we investigated ferrokinetics, RBC life span, karyotype, serum Epo (erythropoietin) concentration, response to therapy and prognosis. Results were as follows: 1) all three RA patients who were younger than 30 years old were included in atypical RA group, 2) in ferrokinetics study PID (plasma iron disappearance time) values of MDS4 and MDS6 patients ranged between those of AA1 and those of MDS3 patients (5 of 7 patients), 3) two cases who developed leukemia belonged to typical RA group, 4) patients with atypical RA showed response to therapy and their prognosis were better than those with typical RA. These observations suggest that atypical RA have different clinical features from typical RA.
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PMID:[Clinical features of atypical refractory anemia (RA)]. 194 25

A 17 year old male was admitted because of pancytopenia. Bone marrow aspiration revealed myelodysplasia, no increase of blast cells and excessive expansion of megakaryocytic lineage. Although mild increase of bone marrow reticulin fiber was observed, no hepatosplenomegaly was recognized. Therefore he was diagnosed as refractory anemia (RA) or MDS with myelofibrosis and treated with low dose Ara-C regimen. Remission was achieved in June 1987, but the relapse occurred in Oct. 1987. His bone marrow at the relapse showed more remarkable dysplastic change than before. Sequential bone marrow examinations thereafter, revealed an increase of megakaryocytic lineage, especially immature dysplastic megakaryocytes, leading to the appearance of the abnormal megakaryoblasts (detected with anti GP IIb/IIIa antibody) as well as uncharacterized blast cells in his terminal stage. Transformation from MDS to megakaryocytic leukemia was strongly suggested. He died of severe pneumonia in March 1989. The invasion of abnormal immature megakaryocytic cells including megakaryoblasts was observed in liver, spleen and lymph nodes at autopsy. There are several reports on cases having a common hematological features such as 1) pancytopenia in peripheral blood, 2) myelodysplasia, 3) excessive growth of megakaryocytic lineage, 4) myelofibrosis without hepatosplenomegaly, although other clinical features were different. We propose all these cases should be reviewed at the point of MDS mainly involved in megakaryocytic lineage.
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PMID:[Myelodysplasia predominantly involving in megakaryocytic lineage successfully treated with low-dose Ara-C]. 194 32

A recombinant human interferon-gamma (IFN-gamma) was added to the clonal and suspension cultures of bone marrow (BM) cells from three patients with refractory anemia with excess of blasts (RAEB) before and after IFN-gamma therapy. IFN-gamma inhibited the hematopoietic progenitor cell growth from normal BM in one patient. In the remaining two patients with RAEB, IFN-gamma at lower concentrations (10-10(2) U/ml) consistently enhanced the growth of hematopoietic progenitors. The addition of IFN-gamma to suspension cultures promoted cellular differentiation in all three cases, as evidenced by persisting cytogenetic changes, Auer-rod containing maturing cells and cytochemical studies. Although IFN-gamma therapy resulted in partial or minor responses, these findings suggest the potential of IFN-gamma to act as an inducer of differentiation in myelodysplastic syndromes both in vitro and in vivo.
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PMID:Interferon gamma induces differentiation of cells from patients with myelodysplastic syndromes in vitro and in vivo. 194 31

In 1987, a 50-year-old patient presented with isolated thrombocytopenia (27,000/mm3) which proved to be refractory to steroid medication and high i.v. doses of immunoglobulin. Two years later he developed macrocytic anemia. Chromosomal analysis confirmed the diagnosis of myelodysplastic syndrome (MDS), refractory anemia type with blast excess. Cytogenetically, three cellular populations were observed: one normal (75% of metaphases) and two abnormal, clone A (2%) 46,XY, del(5q), del(11q), and clone B (23%) 46,XY, del(5q), del(11q) plus 2 other anomalies. Evolution was characterized by worsening of the bicytopenia with marked hypoplasia of the megakaryocytic and erythroid series while the percentage of blasts remained stable. Concerning the chromosomal markers, the normal population disappeared and clone A became predominant (clone A 97%, clone B 3%). This case shows that isolated thrombocytopenia can be the sole initial manifestation of MDS. We discuss the possibility that "refractory thrombocytopenia" constitutes a diagnostic category like refractory anemia or refractory anemia with ring sideroblasts. The proliferative advantage of clone A or the disadvantage of clone B may be due to the occurrence of new, cytogenetically non-detectable mutations.
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PMID:[Unusual initial manifestation in a case of refractory anemia with excess of blasts]. 194 47

A case of acute eosinophilic leukemia (EoL) that occurred in a patient with preexistent myelodysplastic syndrome is reported. The patient was initially diagnosed as having refractory anemia (RA) on the basis of pancytopenia with dysplasia and chromosomal abnormalities. Two years later, he was readmitted because of progression of pancytopenia, and bone marrow and peripheral blood showed immature dysplastic eosinophils. Clonal assay of peripheral blood mononuclear cells revealed autonomous growth of colony-forming unit eosinophils. Cytotoxic chemotherapy did not induce remission, and extensive myelofibrosis developed. Cytogenetic analysis in the RA state showed +1p- and -7 whereas complicated abnormalities including +1p-, 3q- and 7p- dominated in the EoL state.
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PMID:Acute eosinophilic leukemia in a patient with preexistent myelodysplastic syndrome. 195 Mar 62

Circulating myeloid progenitors were assayed in 172 normal subjects and 147 patients with myelodysplastic syndrome (MDS). Patients whose cultures had colony/cluster ratios (CCR) less than 0.3 had significantly shorter survival periods than comparable patients with CCR greater than 0.3. A second prognostic indicator, which complemented CCR, was identified in patients with less than 5% blasts. Median survival was significantly reduced in patients with greater than 15 clusters/ml blood despite colony and cluster numbers being predominantly within the normal range. Characteristic differences were found in three FAB groups large enough to allow statistical analysis. Survival amongst patients with refractory anaemia with excess of blasts (RAEB) was related to CCR and was independent of cluster number. Amongst sideroblastic patients (SA) survival related only to cluster number. Refractory anaemia (RA) patients included individuals in both high-risk groups with only three patients out of 64 showing both features. Amongst all the MDS patients, those with CCR greater than 0.3 and less than 15 clusters/ml blood formed a low-risk group (n = 60) with a relatively good prognosis of whom 85% survived the study period (median duration 938 d) including 94% of those in this group with less than 5% marrow blast cells.
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PMID:Colony-cluster ratio and cluster number in cultures of circulating myeloid progenitors as indicators of high-risk myelodysplasia. 201 53

A 29 year-old-man who had been diagnosed as having myelodysplastic syndrome (MDS) in August 1985 was re-admitted to our hospital because of fever and palpitation. His peripheral blood showed severe pancytopenia and bone marrow findings remained to be compatible with MDS (refractory anemia), but karyotype of bone marrow cells revealed 7 monosomy in 17 of 20 metaphases examined. Other laboratory findings revealed decreased serum haptoglobin, positive urine hemosiderin and the normal resistance of red cell membrane. In addition, both Ham test and sugar water test were negative. The titer of cold agglutinin was low, Donath-Landsteiner antibody was not detected. These findings suggested the association of autoimmune hemolytic anemia (AIHA), although both direct and indirect Coombs' test were negative. After administration of 50mg of prednisolone daily, the frequency of red cell transfusion was markedly decreased and transfused red cell life span was prolonged from 10.4 days to 27 days. Afterward, his hematological status rapidly transformed into that of acute nonlymphocytic leukemia about 13 months after admission and he died of gastrointestinal bleeding and cerebral bleeding. Cases of MDS with immunological disorder have been reported. This is, however, the first case of MDS associated with Coombs negative AIHA.
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PMID:[Coombs negative autoimmune hemolytic anemia in a patient with myelodysplastic syndrome]. 202 40

As anemia is frequently the main problem in myelodysplastic syndromes (MDS), we studied the efficacy of human erythropoietin (rhEpo) in stimulating the erythroid lineage in 14 patients, starting with 40 U/kg three times a week and doubling the dose every 6 weeks until a response was observed. The highest doses administered were 80 (n = 1), 160 (n = 4), 320 (n = 8) and 640 U/kg (n = 1). One patient (refractory anemia with an excess of blasts, RAEB) showed an increase of hemoglobin, white blood cells and platelets with 80 U/kg rhEpo. However, this patient developed acute leukemia while on therapy. Two other patients (RAEB and RAEB in transformation) also transformed to acute leukemia. In the other 11 patients no response was observed. There was no correlation between in vitro culture data and in vivo responsiveness. The treatment was well tolerated and no nonhematological side effects were observed. From this study we conclude that rhEpo, even when given at high doses, has a low response rate in patients with MDS. Further investigation is needed in order to clarify whether rhEpo increases the potential risk of transformation to acute leukemia.
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PMID:Recombinant human erythropoietin in patients with myelodysplastic syndromes. 203 64

A 64-year-old man had urticaria pigmentosa and myelodysplasia (refractory anemia with excess blast cells; partial trisomy 8 syndrome) without increased numbers of marrow mast cells. Clonal marrow assays in agar demonstrated normal to increased colony-forming units of granulocytes/macrophages. In long-term liquid cultures containing mast cell growth factor (interleukin 3), his marrow cells proliferated after 3 weeks to produce abnormal myeloid precursors similar to those in the corresponding marrow aspirate specimen. Cells with basophilic-staining granules were less abundant in comparison with normal marrow specimens cultured similarly. These results suggest that the mast cells in this patient are not of the same clone as the preleukemic marrow cells, although the possible marrow-cell origin of urticaria pigmentosa mast cells cannot be excluded. Previous reports suggest that urticaria pigmentosa without systemic mastocytosis occurs as a nonspecific abnormality in a variety of myeloid, lymphoid, and nonhematologic malignancies. Our data also support this hypothesis that urticaria pigmentosa is a reactive process rather than a manifestation of clonal proliferation of the primary malignancy.
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PMID:Urticaria pigmentosa and preleukemia: evidence for reactive mast cell proliferation. 205 Aug 59

A series of 27 patients with myelodysplastic syndrome (MDS) was analysed to determine the clinical and pathologic features, the natural history and the prognostic factors. The clinical features of these patients included the following: mean age 53.77 years; symptoms due to cytopenias 100 per cent; hepatomegaly 44.5 per cent; splenomegaly 11 per cent. Almost all patients with MDS presented anemia; additional cytopenias were present in many patients. The bone marrow was hypocellular in 1/3 of cases. The patients have been classified according to the French-American-British (FAB) Group criteria: there were 11 patients with refractory anemia, 11 patients with refractory anemia with sideroblasts and 5 patients with refractory anemia with excess of blasts. The mean survival was 19 months, 9 patients died: death was due to infection or hemorrhage in 7 cases and to evolution to acute myelogenous leukemia (AML) in 2 cases (10.5%). Thrombocytopenia, the Bournemouth scoring system and FAB classification were independent prognostic factors.
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PMID:[Myelodysplastic syndromes. A clinico-biological analysis of 27 cases]. 207 37


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