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

We have identified an identical reciprocal translocation between the long arms of chromosomes 3 and 21 with breakpoints at bands 3q26 and 21q22, [t(3;21)(q26;q22)], in the malignant cells from five adult patients with therapy-related myelodysplastic syndrome (t-MDS) or acute myeloid leukemia (t-AML). Primary diagnoses were Hodgkin's disease in two patients and ovarian carcinoma, breast cancer, and polycythemia vera in one patient each. Patients had been treated with chemotherapy including an alkylating agent for their primary disease 1 to 18 years before the development of t-MDS or t-AML. We have not observed the t(3;21) in over 1,500 patients with a myelodysplastic syndrome or acute myeloid leukemia arising de novo or in over 1,000 patients with lymphoid malignancies. We have previously reported that the t(3;21) occurs in Philadelphia chromosome-positive chronic myelogenous leukemia (CML). Thus, the t(3;21) appears to be limited to t-MDS/t-AML and CML, both of which represent malignant disorders of an early hematopoietic precursor cell. These results provide a new focus for the study of therapy-related leukemia at the molecular level.
Blood 1990 Dec 15
PMID:t(3;21)(q26;q22): a recurring chromosomal abnormality in therapy-related myelodysplastic syndrome and acute myeloid leukemia. 226 51

The myelodysplastic syndromes are bone marrow stem cell disorders that result in disorderly and ineffective hematopoiesis. They are prognostically heterogenous. Approximately one third of cases evolve to acute myeloid leukemia. Many additional cases terminate in severe bone marrow failure. The French-American-British Working Group classification of the myelodysplastic syndromes defines morphologic and prognostic groups. Cytogenetic and in vitro cell culture characteristics are important prognostic indicators.
Clin Lab Med 1990 Dec
PMID:Diagnosis, classification, and course of myelodysplastic syndromes. 227 70

Clonal chromosome abnormalities are found in more than half the patients with hematologic malignancies. Karyotype is an independent prognostic factor in these patients. Cytogenetic findings correlate significantly with morphologic, immunologic, and clinical features as well as response to treatment, remission duration, and survival. The number of different cytogenetic abnormalities is enormous; however, many cytogenetic findings frequently occur in a given disease (e.g., abnormalities of 5 or 7 in 75% to 90% of patients with therapy-related AML). Some abnormalities are found only in myeloid malignancies, for example, the t(8;21)(q22;q22) and rearrangements of chromosome 16q22, both of which have a good prognosis. Other abnormalities usually are found in both myeloid and lymphoid malignancies, for example, the t(4;11)(q21;q23) and t(9;22)(q34;q11), both of which have a poor prognosis. The Human Gene Mapping Conferences have compiled much cytogenetic data and produced several interesting correlations in myeloid malignancies: rearrangements of 3q21-26 with myeloid proliferations associated with environmental exposure (similar to abnormalities of 5q, 7q, 12p, and 17q), aberrations of 12p, 11q13 and 11q23 with both myeloid and lymphoid disorders, and the lack of myeloid involvement and abnormalities of chromosomes 14 and 18. In conclusion, cytogenetic analysis of neoplastic cells at diagnosis for patients with MDS, AML, and SAML is required for appropriate diagnosis and treatment. The use of chromosome abnormalities to separate patients into high- and low-risk groups eventually may allow us to be more effective in selecting curative therapy.
Clin Lab Med 1990 Dec
PMID:Chromosomal abnormalities in myelodysplastic syndromes and acute myeloid leukemia. 227 73

Mutations in the ras genes frequently are found in myeloproliferative disorders. These abnormalities have prognostic significance in the myelodysplastic syndromes, but their clinical importance in other hematopathic states remains unclear. The study of this gene in leukemic conditions is providing insights into the genetic pathways leading to leukemic progression.
Clin Lab Med 1990 Dec
PMID:The role of ras gene mutations in myeloproliferative disorders. 227 74

The active metabolite of vitamin D known as 1,25-dihydroxyvitamin D3 [1,25(OH)2D3] is a major physiologic regulator of mineral metabolism in man. The compound is also a potent inducer of differentiation of a human promyelocytic leukemia cell line known as HL-60. The induction of differentiation of myeloid leukemia cells to functional end cells offers an appealing therapeutic prospect. We investigated the ability of 1,25(OH)2D3 both to induce in vitro the differentiation of blast cells taken from patients with acute myelogenous leukemia and to improve hematopoiesis in vivo in patients with the myelodysplastic syndromes (preleukemia). We found that high concentrations (10-6 M) of 1,25(OH)2D3 significantly induced the in vitro differentiation of blast cells as measured by morphology, phagocytosis, and superoxide production. A concentration of 10-9 M 1,25(OH)2D3 had no effect on blast cell differentiation. We gave 2 microgram/day of 1,25(OH)2D3 to 18 patients with myelodysplastic syndrome (preleukemia) in an attempt to improve their hematopoiesis. During therapy, their peak peripheral blood granulocyte, platelet, and macrophage concentrations were slightly elevated as compared to their baseline, starting levels. Eight patients had a partial or minor peripheral blood response to the compound during the administration of 1,25(OH)2D3. However, no patient showed significant improvement of peripheral blood cell or marrow blast cell counts by the end of the study (greater than or equal to 12 weeks) as compared to their starting levels. Seven of the patients developed leukemia before or by 12 weeks of treatment. Nine of the 18 patients developed hypercalcemia. Taken together, the study shows that high concentrations (10-6M) of 1,25(OH)2D3 can induce differentiation of leukemia blast cells in vitro, but the administration of 1,25(OH)2D3 to patients with the myelodysplastic syndromes (preleukemia) does not have an enduring therapeutic effect. Hypercalcemia prevented administering greater amounts of 1,25(OH)2D3. In the future, the use of new vitamin D analogs that induce hematopoietic cell differentiation without inducing hypercalcemia might allow the achievement of higher blood levels of the inducing compound and might be medically useful for selected preleukemic and leukemic patients.
Cancer Treat Rep 1985 Dec
PMID:1,25-Dihydroxyvitamin D3: in vivo and in vitro effects on human preleukemic and leukemic cells. 241 38

I believe meningomyelocele is not vanishing, but is a new and diminishing disorder. Prenatal diagnosis will be accepted only by a portion of most communities if abortion is the only alternative to the delivery of an impaired child. I believe participation in neural tube screening programmes can be increased by offering improved pregnancy outcome with concomitant prelabor caesarean section in addition to termination. Regardless, the prevalence at birth of children with myelomeningocele will decrease both in total numbers and in the severity of the expressed lesion. The resultant rarity will require collaboration between centers to evaluate treatment. The developing International Myelodysplasia Study Group using a Patient Data Management System and computer-assisted analysis is a model of successful collaboration that allows better exploration of the multiple variables that contribute to the well-being of children with open neural tube defects of the spine.
Z Kinderchir 1986 Dec
PMID:Meningomyelocele: a new or a vanishing disease? 243 50

The colony-stimulating factors (CSFs) are polypeptide hormones that regulate myeloid proliferation and differentiation. Granulocyte-macrophage CSF (GM-CSF) has been used in leukopenic acquired immunodeficiency syndrome (AIDS) patients, myelodysplastic syndrome patients, and cancer patients after intensive chemotherapy. Its hematologic activity results in an increase in neutrophil, monocyte, and eosinophil counts. Recombinant GM-CSF effectively restores granulopoiesis in AIDS patients and appears capable of improving myelopoiesis and, in some cases, erythropoiesis in subjects with myelodysplastic syndrome. It accelerates bone marrow recovery after chemotherapy. Similarly, granulocyte CSF has demonstrated the capacity to reduce the granulocyte nadir after single agent or combination chemotherapy. Thus, CSFs have considerable promise for therapy for bone marrow failure states.
Semin Oncol 1988 Dec
PMID:Clinical applications of colony-stimulating factors. 246 48

We studied the effects of heparin, dextran sulphate (MDS), gabexate mesilate (FOY), nafamostat mesilate (FUT-175) and argipidine (MD-805) on APTT, PT, thrombin time (TT) and kaolin-activated PTT (KPTT) with various concentrations of phospholipid for screening of lupus anticoagulants (LA). Heparin, MDS and FUT-175 had a greater effect on APTT than PT. On the contrary, MD-805 had a similar effect on both APTT and PT, which suggests that MD-805 inhibits thrombin generation equally on intrinsic and extrinsic coagulation pathways. Heparin and MD-805 were more effective on TT than MDS, FUT-175 and FOY at high concentrations significantly prolonged TT. But, at even higher concentrations of FUT-175, prolongation of TT was reduced contrary to our expectation. With FOY TT became less prolonged with a passage of time, suggesting time-dependent reduction of its anticoagulant activity. Heparin (0.1-0.2 U/ml) and MDS (0.1-0.3 mg/ml) did not have any effect on KPTT with high concentration of phospholipid, but did FUT-175. It suggests that phospholipid inhibits anticoagulant activity of heparin and MDS. Anti-phospholipid activity of heparin and MDS is similar to that of LA. We concluded that the differentiation of LA from heparin-like inhibitors is needed.
Rinsho Byori 1989 Dec
PMID:[The effects of various anticoagulants on blood coagulation: with special reference to false positive lupus anticoagulants]. 251 25

The clonal composition of each cell population was determined from the characteristic methylation pattern of DNA and the restriction fragment length polymorphism (RFLP) of the hypoxanthine phosphoribosyltransferase (HPRT) and phosphoglycerate kinase (PGK) genes, both located on the X chromosome. About 71% of Japanese females are heterozygous in terms of the RFLP of either HPRT or PGK genes, which was demonstrated by using 5' genomic DNA or cDNA probes for these genes. All 3 cases of chronic myeloproliferative disorders showed monoclonal patterns. AML or ALL cases demonstrated either monoclonal or polyclonal patterns depending upon the percentage of blastic cells. Monoclonal patterns were seen in 3 of 4 cases of myelodysplastic syndromes and both PNH cases.
Nihon Ketsueki Gakkai Zasshi 1989 Dec
PMID:Molecular genetic approach to the analysis of clonal proliferation in hematologic disorders. 257 94

Clinical, cytomorphologic, and cytogenetic investigations were carried out in a series of 76 secondary MDS and ANLL. Chromosome abnormalities were more frequent in patients with a history of multiple myeloma or macroglobulinemia (92%) and myeloproliferative disorders (82%) than in patients with previous breast cancer (40%). The secondary hematologic malignancies were mostly a trilineage bone marrow disorder. The most commonly found cytogenetic anomaly was monosomy 7, followed by total or partial loss of chromosome 5. In addition six other chromosomes, i.e., chromosome 3, 8, 9, 12, 17, and 21 seemed to be consistently involved in the pathogenetic mechanisms of secondary leukemia and MDS.
Cancer Genet Cytogenet 1989 Dec
PMID:Cytogenetic and clinical investigations in 76 cases with therapy-related leukemia and myelodysplastic syndrome. 259 67


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