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)

Recently, a novel approach has been used in the treatment of leukemia: induction of the leukemic cells to undergo terminal differentiation. Based on its in vitro ability to induce differentiation in several myeloid leukemic cell lines, retinoic acid (RA) has been applied clinically in cases of myelodysplastic syndromes and acute myeloid and promyelocytic leukemia. In the present study we have determined in detail the ability of RA to induce expression of granulocytic functions in a human promyelocytic leukemia cell line (HL-60) and compared it with that of dimethylsulfoxide (DMSO). Several granulocytic characteristics (phagocytosis, surface adherence and generation of free radicals in response to phorbol-ester) were induced to the same degree by both agents. Other normal neutrophil functions, including lysozyme accumulation, spontaneous migration, chemotactic activity toward zymosan-activated serum (containing C5a), the peptide N-formyl-methionyl-leucyl-phenylalanine (FMLP) and spontaneous motility in semi-solid medium were induced by DMSO, but they were absent or incompletely expressed in RA-induced cells. In contrast, only RA induced migration toward leukotriene B4 (LTB4). Simultaneous treatment with RA and DMSO proved synergistic with respect to morphological maturation and several functions (e.g. NBT reduction), but complementary stimulation of other activities (e.g. chemotaxis, lysozyme content) could not be demonstrated. Furthermore, characteristics induced by DMSO (i.e., expression of C5a and FMLP receptors and accumulation of lysozyme) were inhibited by the addition of RA.(ABSTRACT TRUNCATED AT 250 WORDS)
Cell Differ 1987 Sep
PMID:Expression of granulocytic functions by leukemic promyelocytic HL-60 cells: differential induction by dimethylsulfoxide and retinoic acid. 347 6

Two patients with acute myelomonocytic leukemia (AML-M4) and a specific chromosomal translocation t(6;9)(p23;q34) are reported and compared to 21 AML patients with the same translocation collected from the literature. Our observation suggest that AML with t(6;9)(p23;q34) is characterized by myelodysplasia, basophilia, and a variety of blast cell morphologies (M1, M2, M4) with a greater proportion of the cases than previously appreciated being examples of acute myelomonocytic leukemia (AML-M4). The consistent association of myelodysplasia provokes the proposal that this subtype of de novo AML is a result of an acute stem cell disorder. The poor outcome with standard AML chemotherapy experienced in this group of relatively young patients necessitates consideration of alternative therapeutic strategies such as early bone marrow transplantation.
Am J Hematol 1987 Sep
PMID:Acute myelomonocytic leukemia (AML-M4) and translocation t(6;9)(p23);q34): two additional patients with prominent myelodysplasia. 347 96

The synthetic oligonucleotide probes were used for the analysis of N-ras oncogenes detected in human acute leukemias. The mutations of N-ras genes were observed to occur randomly among the subtypes of myeloid leukemias, whereas the N-ras mutations at codon 12 are more likely to occur in lymphoid leukemias than other mutations. The mutations at codon 13 of the N-ras gene were not detected in acute leukemias although they were found in myelodysplastic syndrome that is considered to be a preleukemic state.
Biochem Biophys Res Commun 1987 Sep 15
PMID:Activation mechanism of the N-ras oncogene in human leukemias detected by synthetic oligonucleotide probes. 347 29

We present four cases with a malignant hematologic disorder and an inversion (peri- or paracentric) involving chromosome #12. Two cases of myelodysplastic syndrome showed an identical acquired pericentric inv(12)(p12q23). The pertinent literature on constitutional and acquired inversions of chromosome #12 has been revised.
Cancer Genet Cytogenet 1987 Sep
PMID:Inversions of chromosome 12 in human malignancies. 360 33

Leukocyte-derived inhibitory activity inhibiting the entry of normal progenitor cells of granulocytes and macrophages (CFU-GM) into the S-phase of a cell cycle was investigated in 16 patients with different forms of myelodysplastic syndrome (MDS). The presence of this inhibitory activity was analysed in medium conditioned with low-density cells obtained from peripheral blood of MDS patients. The inhibition rate was measured by 3H-thymidine suicide technique with subsequent cultivation of pretreated cells in semisolid agar medium. Low-density cells from MDS patients of various types were studied: from the twelve patients with refractory anaemia (RA or RAS) only three were positive, one patient with chronic myelomonocytic leukaemia (CMML) was negative while one patient with refractory anaemia with excess of blasts (RAEB) and two patients with RAEB in transformation (RAEB-T) were positive with respect of the described test. In two patients with RA, who underwent a long-term investigation, the production of leukocyte-derived inhibitory activity preceded the development of disease into RAEB or RAEB-T. In five positive cases, supernatants were incubated with antiserum against human placental ferritin; with one exception, the inhibitory activity was neutralized.
Blut 1987 Sep
PMID:Leukocyte-derived inhibitory activity in patients with myelodysplastic syndrome. 362 Jul 12

A review of 129 consecutive young patients (average age 13.4 years) who underwent intestinocystoplasty or total bladder replacement during a 6 1/2-year period at our institution is presented. The most common diagnosis was myelodysplasia and the average followup was 44 months. The clinical and urodynamic aspects of 4 types of intestinocystoplasty (ileocecal, tubular sigmoid, sigmoid patch and ileal patch) are presented. All 4 operations resulted in a significant increase in bladder volume, with a decrease in filling pressure and, thus, improved vesical compliance. In combination with clean intermittent catheterization renal function was maintained or improved in 91 per cent and urinary continence was achieved in 82 per cent of the patients. Hyperchloremic acidosis requiring therapy was noted only in patients with pre-existing renal insufficiency, although mild hyperchloremia after cystoplasty was seen with all 4 types of cystoplasty. There were no operative mortalities. Significant surgical complications occurred in 36 per cent of the patients, the most common of which was vesicoureteral reflux in the ileocecal cystoplasty. Mass unit peristaltic contractions occurred in 34 per cent of the tubular-shaped bowel segments compared to only 10 per cent of the patch segments. These peristaltic contractions contributed to the greater postoperative morbidity noted in the tubular large bowel cystoplasties. The over-all success rate for intestinocystoplasty in this series was 84 per cent. Intestinocystoplasty is an effective procedure when used to increase the compliance of the lower urinary tract. In combination with clean intermittent catheterization it can be applied successfully to patients with neurogenic bladder dysfunction. Large and small bowel seem to have similar clinical and urodynamic properties. The type of intestinal segment used for intestinocystoplasty seems to be of less importance than the size and configuration. Large bowel in its native tubular configuration should be avoided.
J Urol 1987 Sep
PMID:Intestinocystoplasty and total bladder replacement in children and young adults: followup in 129 cases. 362 61

Seventy-nine newborns with myelodysplasia were followed up for 1 1/2 to six years with serial roentgenographic, neurologic, and urodynamic evaluations. Twenty-nine infants (37%) demonstrated changes in external urethral sphincter innervation during the first three years of life. Of the group who changed, 896% (25/29 infants) did so in the first year, 10% (3/29 infants) in the second year, and 4% (1/29 infants) in the third. The risk factor for change was 32%, 6%, and 2%, respectively, during each of these three years. Fifteen infants (19% of the total) experienced neurourologic deterioration while 14 infants (18%) improved. Seven newborns underwent a second neurosurgical procedure resulting in improvement in four, stabilization in two, and further deterioration in one. These findings underline the importance of early and continued neurologic and urologic surveillance in children with myelodysplasia because it is a dynamic disease process.
JAMA 1987 Sep 25
PMID:The changing neurourologic lesion in myelodysplasia. 362 70

Patterns of erythropoiesis and granulopoiesis were studied in 15 patients with myelodysplastic syndromes and in one with smouldering leukaemia by correlating CFU-C, BFU-E and CFU-E cloning efficiency with erythroid and myeloid maturation indices derived from quantitative 14C-autoradiography and with ferrokinetics. Maturation index of a cell lineage was defined as the ratio of cell production rate increase from the first to the last proliferative compartment over the corresponding normal value. The myeloid maturation index was reduced in all cases, but CFU-C progenitor frequency was increased by a factor of 3. Erythroid maturation index was also reduced in most cases, and BFU-E progenitor frequency was reduced by a factor of 2. Similarly, CFU-E cloning efficiency corrected for the erythroid maturation index was 2.5-10-fold lower than normal. Comparison of the erythroid maturation index with ferrokinetics revealed a constant ratio of ineffective erythropoiesis with a maturation disturbance in the proliferative pool twice that in the non-proliferative pool. These findings indicate basic qualitative and quantitative abnormalities and a difference in the patterns of production of erythroid and myeloid cells in the myelodysplastic syndromes: Myeloid progenitors are increased in number but their maturation is grossly abnormal. On the other hand, the apparent reduction in erythroid progenitors and the reduced erythroid maturation index are attributable to both premature cell death of the more mature erythroblasts and an increased proerythroblast proliferation, i.e. extra divisions in the proerythroblast compartment at the expense of maturation.
Br J Haematol 1987 Sep
PMID:Erythroid and myeloid maturation patterns related to progenitor assessment in the myelodysplastic syndromes. 366 22

A case is reported of a young man with cardiomyopathy and myelodysplasia with bone marrow and peripheral blood eosinophilia. Cytogenetic investigation revealed a (5;16) (q33;p13) translocation. This is the first report of myelodysplasia with eosinophilia associated with a break in 16p13 alone, with no abnormality of 16q22.
Leukemia 1987 Sep
PMID:A myelodysplastic syndrome with eosinophilia associated with a break in the short arm of chromosome 16. 366 76

147 patients with myelodysplastic syndromes were investigated for the presence of micronuclei and nuclear budding in bone marrow erythroblasts. The patients were divided into subgroups on the basis of bone marrow karyotype, 31 healthy bone marrow donors constituted a control group. Patients with monosomy 7 or 7q- and patients with major karyotypic abnormalities (MAKA) had significantly more erythroblasts with micronuclei and nuclear budding than the control group. Patients with a 5q- chromosome as the sole karyotypic aberration had more micronuclei than the controls. For other patients with MDS the differences were statistically nonsignificant.
Eur J Haematol 1987 Sep
PMID:Correlation between bone marrow karyotype and the occurrence of erythroblast micronuclei and nuclear budding in patients with myelodysplastic syndromes. 367 75


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