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

We report a series of 39 cases of acquired idiopathic sideroblastic anemia accounting for 12 p. 100 of the 330 cases of myelodysplastic syndrome diagnosed in our department in six years. Patients' mean age was high (70.8 years). Anemia was present in 92 p. 100 of the cases and was of the macrocytic type in 87 p. 100 of the patients. Only two patients (5 p. 100) had neutropenia and two (5 p. 100) had thrombocytopenia. Thrombocytosis was observed in 17 cases (43 p. 100); it was usually moderate. The mean proportion of medullary ring-sideroblasts was 35 +/- 14 p. 100. A clonal abnormality was found in 4 of the 11 karyotype examinations performed. The course of the disease was marked by recurrences of anemia which required regular blood transfusions in 82 p. 100 of the cases. In at least 30 p. 100 of the patients followed up for more than 30 months, these transfusions were responsible for secondary haemochromatosis. Conversely, in only three patients (7.5 p. 100) the disease progressed to acute leukemia. On the other hand, 4 patients (10 p. 100) developed thrombocythemia (greater than 1,000 x 10(9)/1). Median actuarial survival rate was 48 months. There were only two factors of poor prognosis: age over 70 and anemia with less than 8 g/dl haemoglobin at the time of diagnosis. Transfusion haemochromatosis is the principal risk in acquired idiopathic sideroblastic anemia: this risk can be prevented by iron chelating agents in patients with frequent blood perfusions.
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PMID:[Acquired idiopathic sideroblastic anemia. Apropos of 39 cases]. 279 16

In 98 patients with chronic myeloproliferative disorders (45 chr. myeloid leukaemia (CML), 19 myelofibrosis primaria (MP), 28 polycythaemia vera (PV) and 6 idiopathic thrombocythaemia (IT)) the incidences of increased numbers of MPO-deficient polymorphonuclear (PMN) were 60% in CML, 32% in MP, 7% in PV and 0% in IT patients. The CML figure differed significantly from the others (p less than 0.001). This study confirms the finding of low NAP scores in CML compared to normal or high NAP scores in the other groups of the myeloproliferative syndrome. The incidences of increased numbers of MPO-deficient PMN in this study are comparable to those found in the primary myelodysplastic syndromes and in acute myeloid leukaemia. The finding supports the view that some of the CML cases and may be other cases of the chronic myeloproliferative disorders may be fundamentally the same disease as in primary myelodysplastic syndromes and in acute myeloid leukaemias.
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PMID:Myeloperoxidase-deficient polymorphonuclear leucocytes (VII): Incidence in untreated myeloproliferative disorders. 300 24

Three patients with chronic red cell aplasia also showed thrombocytosis or granulocytosis, or both. All had morphological evidence of myelodysplasia on examination of bone marrow aspirate but none had a detectable chromosomal abnormality. These patients seem to provide evidence of a separate entity within the spectrum of myelodysplastic and myeloproliferative disease.
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PMID:Red cell hypoplasia, thrombocytosis, and leucocytosis: myelodysplastic and proliferative syndrome. 250 80

This paper analyzes the hematologic features and outcome of 13 patients with chromosome 5 abnormalities (monosomy 5 or deletion of 5q), either isolated or with additional anomalies. Among four patients with isolated del (5q), two had a stable refractory macrocytic anemia with thrombocytosis (5q-syndrome). All nine patients with complex karyotypes had acute leukemia or refractory anemia with excess of blasts in acute transformation; two cases were TdT-positive, with a lymphoid or a mixed phenotype. In seven patients, preleukemia preceded overt leukemia, and in six, a prior therapeutic, or occupational exposure to mutagens/carcinogens had occurred. Additional chromosome 7 abnormalities were seen in four cases. The median survival of patients with complex karyotypes was 19 months from the time of diagnosis of the hematologic disorder and 5 months from the time of identification of the chromosome 5 abnormality. Pathogenetic implications of the chromosome 5 monosomy or del (5q) through a proto-oncogene activation and the putative hemopoietic stem cell involvement in a clonal disease are discussed.
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PMID:Hematologic and clinical features of patients with chromosome 5 monosomy or deletion (5q). 335 40

A patient developed a secondary blood disorder 7 years after radiotherapy for a gastric lymphoma. The initial myelodysplastic syndrome evolved to a myeloproliferative phase with transient polycythemia, progressive thrombocythemia, and hyperleukocytosis. Chromosome analysis performed in the terminal phase showed del(5)(q13q31),t(9;22)(q34;q11), and a complex rearrangement involving chromosomes #2 and #3. A correlation between chromosomal abnormalities and hematologic findings could be established. In this case, we have assumed that the Philadelphia translocation is a late event, due to prior mutagen exposure, and its association with a common secondary abnormality (5q-), followed by a progressively developing myeloproliferative phase. Furthermore, the association of Ph and 5q- in a single clone seems to indicate that the same stem cell is affected by these two abnormalities.
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PMID:Association of the Philadelphia chromosome and 5q- in secondary blood disorder. 342 79

The term myeloproliferative disease may be applied to all the non-lymphoid dysplastic and neoplastic conditions arising from the haematopoietic stem cell or its progeny. Thus the chronic and acute myeloid leukaemias, thrombocythaemia, megakaryocytic myelosis, myelofibrosis, the myelodysplastic syndromes and some cases of aplastic anaemia may be viewed as variants of a single disease process. This view is useful in explaining the common occurrence of mixed forms of disease or interconversions between the myeloproliferative diseases. This variability is a consequence of the development of all the haematopoietic lineages from a single class of haematopoietic stem cell by progressive differentiation. The aetiology of the myeloproliferative diseases in the domestic animals is uncertain but feline leukaemia virus infection has been implicated in the cat. These conditions may be classified as aplastic anaemia, as preleukaemic dysplastic conditions with variable cytopenias and morphological abnormalities of blood cells, as smouldering leukaemias, or as leukaemias with a frankly leukaemic blood or bone marrow.
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PMID:Myeloproliferative disease in the dog and cat: definition, aetiology and classification. 342 14

A unique patient with sideroblastic anemia and thrombocytosis with a recurrent and ultimately fatal thromboembolic phenomenon is described. Cytogenetic analysis of bone marrow metaphases revealed a pseudodiploid chromosome complement, 46,XY,ins(3;3)(q26;q21q26). The association of thrombocytosis and ins(3;3) in patients with preleukemia or myelogenous leukemia has been reported previously. The association of ins(3;3) and thrombocytosis in our patient with sideroblastic anemia suggests that the disorder may involve a hematopoietic progenitor cell capable of giving rise to all three cell lineages. Our findings also support the suggestion that a gene on the long arm of chromosome #3 may encode a protein that, at least in part, regulates megakaryopoiesis.
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PMID:Sideroblastic anemia associated with thrombocytosis and a chromosome 3 abnormality. 345 20

Ten patients with a hematologic disorder and a clone of cells with trisomy 9 in the bone marrow were studied in order to investigate the clinical significance of this chromosome anomaly. In five of the patients, trisomy 9 was the only anomaly; in four, there was also trisomy 8; and in one, a Y chromosome was also lacking. Four patients had a myelodysplastic syndrome, and six had a myeloproliferative disorder. Interestingly, four patients had primary thrombocytosis.
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PMID:Trisomy 9 in hematologic disorders: possible association with primary thrombocytosis. 347 49

N4-Palmitoyl-1-beta-D-arabinofuranosylcytosine (PL-AC) was administered p.o. to 199 patients with acute leukemia, myelodysplastic syndromes (MDS) and myeloproliferative disorders (MPD). Of 76 patients with AML, 11 achieved complete remission (CR) and 7 achieved partial remission (PR). Of 8 patients with ALL, 2 achieved CR and 1 achieved PR. Of 3 patients with blast crisis of MPD, 1 achieved CR. CR was reached with PL-AC at 100-900 mg/day after 5-98 (median 26) days. Of 50 patients with MDS, 2 achieved CR, 2 showed good response and 7 partial response. Response was reached with 100-400 mg/day after 13-122 (median 32) days. Improvement of polycythemia vera was observed in 6 of 13 patients, and reduction of thrombocytosis was observed in 20 of 23 patients with essential thrombocythemia and myelofibrosis. Of 18 patients with CML, 1 achieved CR. Major side effects were GI toxicities and myelosuppression. In spite of the disadvantages of the oral form of the drug, such as unpredictable absorption, PL-AC may be useful in the treatment of acute leukemia, especially that of the aged, a condition for which intensive chemotherapy is not always indicated, and MDS, which do not necessarily require admission to a hospital.
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PMID:[A phase II study of N4-palmitoyl-1-beta-D-arabinofuranosylcytosine (PL-AC) in patients with acute leukemia and myelodysplastic syndromes. Cooperative Study Group for PL-AC]. 361 59

The clinical and cell growth characteristics of 11 children with monosomy 7 presenting as preleukemia (eight cases) or acute nonlymphoblastic leukemia (three cases) were studied. Anemia was common to all patients, with nine showing leukocytosis, seven thrombocytopenia, and one thrombocytosis. There was a striking predominance of males (M/F ratio, 10:1) and a young median age (3 years). Preleukemia evolved to acute nonlymphoblastic leukemia in five patients and to myelofibrosis in one. In vitro studies of bone marrow progenitor cells cultured in leukocyte feeder-stimulated agar revealed abnormal cell proliferative patterns, most often an increased number of small clusters, for all 11 subjects. The cells of some preleukemic patients showed increased growth even in the absence of an exogenous source of colony-stimulating factor, suggesting autonomous growth or possibly autocrine stimulation. Combination chemotherapy or bone marrow transplantation failed to induce complete remission in the seven patients who were treated. Our findings in these 11 cases confirm the poor prognosis of monosomy 7 presenting as preleukemia in children. The in vitro studies suggest an association between altered cell growth in vitro and clinical evolution to frank leukemia.
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PMID:Childhood monosomy 7 syndrome: clinical and in vitro studies. 366 40


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