Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023473 (chronic myeloid leukemia)
 18,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We have studied the expression of c-abl and c-myc in leukemic cells of patients in all clinical phases of chronic myelogenous leukemia. We demonstrate that an aberrant 8-Kb c-abl related transcript is present in the RNA of the leukemic cell from all patients with Ph+ CML and that the loss of both normal chromosome #9 is associated with the loss of the normal c-abl related transcripts. This represents direct evidence that the normal c-abl related transcripts derive from the normal c-abl gene locus on the normal chromosome #9, while the aberrant c-abl related transcript in Ph+ CML derives from the hybrid bcr-abl gene formed as a result of the t(9;22). We further demonstrate that trisomy 8 in some instances is associated with enhanced expression of the c-myc oncogene.
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PMID:Molecular characteristics of chronic myelogenous leukemia in blast crisis. 347 57

Two hundred forty-two patients with Philadelphia chromosome-positive chronic myelogenous leukemia in blast crisis were reviewed to identify significant biologic and prognostic associations. Twenty percent of patients had lymphoid blast crisis. Clonal evolution was present in 60 percent of patients at blast crisis and involved most frequently the development of a double Philadelphia chromosome, trisomy 8, or isochromosome 17. The overall median survival from blast crisis was 18 weeks. Patient characteristics demonstrated to have significant association with short survival were: anemia; thrombocytopenia; myeloid or undifferentiated blast cell morphology; clonal evolution involving the presence of a double Philadelphia chromosome, trisomy 8, or isochromosome 17; and low marrow blast percentage. Of 195 patients who received therapy for blast crisis, complete remission was achieved in 44 (23 percent) patients, and 24 (13 percent) patients had a partial remission or hematologic improvement. Lower complete remission rates were associated with old age, thrombocytopenia, myeloid or undifferentiated blast cell morphology, clonal evolution--especially isochromosome 17 and trisomy 8--and long interval from diagnosis to onset of blast crisis. A multivariate analysis identified two characteristics to have independent prognostic importance for both survival and remission: platelet counts and blast cell morphology. In addition, clonal evolution had additive prognostic value for survival (double Philadelphia chromosome) and for response (isochromosome 17). The beneficial association of therapy with survival was demonstrated by the significantly longer median survival of patients treated since 1981 compared with those treated earlier, even after accounting for the pretreatment prognostic factors, and by the significant improvement in survival of patients achieving remission using the "landmark" analysis technique.
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PMID:Chronic myelogenous leukemia in blast crisis. Analysis of 242 patients. 347 58

The findings presented indicate that splenectomy during the blastic phase (BP) of Philadelphia (Ph) positive chronic myeloid leukemia (CML) prolonged survival after the onset of the BP in a group of patients who had, in addition to the Ph, only chromosomally abnormal cells in the marrow (AA patients). To evaluate the predictive parameter for splenectomy in the chronic phase (CP) of CML, the cytogenetic data obtained during the CP of the AA group were compared retrospectively with those of cytogenetically defined groups. Only AA patients showed the presence of abnormal clone(s) containing abnormalities such as trisomy 8, i(17q), and a missing Y chromosome several years before the onset of BP. This may indicate that the presence of these chromosome abnormalities during the CP could be utilized as a predictive parameter for splenectomy in the CP of CML.
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PMID:Chromosome changes and splenectomy in Ph-positive CML. III. Predictive parameters in the chronic phase. 385 66

To evaluate the appearance of chromosome changes, in addition to the Philadelphia (Ph) chromosome, as predictive and diagnostic parameters of transformation in chronic myeloid leukemia (CML), such changes were analyzed in the chronic phase (CP) and compared with those of the blastic phase (BP) of CML. The common chromosome changes observed in the CP were loss of a Y (-Y), trisomy 8 (+8), an isochromosome for the long arm of chromosome #17 [i(17q)], a double Ph (+Ph), reciprocal translocations, and partial deletions. In most patients with chromosome changes in addition to the Ph, the percentage of abnormal clones increased steadily during the CP and was accompanied by other chromosome changes shortly before or at the onset of the BP, except for cases with -Y or i(17q) clones. In general, most chromosome changes observed shortly before or at the BP were complex. These facts suggest that complex chromosome changes could be utilized as predictive and diagnostic parameters of blastic transformation in CML.
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PMID:Chromosomal characteristics of chronic and blastic phases of Ph-positive chronic myeloid leukemia. 385 76

The preleukemic syndrome occurs mainly after middle age. We report 11 patients, aged 62 to 92 years, who presented with weakness, fatigue, malaise and pallor. Eight patients died; survival from the time of diagnosis was between 2 and 21 months. Two of them developed acute myelomonocytic leukemia. A third patient developed Philadelphia chromosome-negative chronic myeloid leukemia within 9 months. Serum unsaturated B12 binding capacity and transcobalamin I were elevated in this patient, preceding the transformation to chronic myeloid leukemia. Five other patients died from sepsis or pneumonia. All patients were anemic, and 10 were leukopenic. Bone marrow was hypocellular in 1 and hypercellular in 10 cases. Chromosomal studies were performed in five patients, with three showing abnormal findings: 47xx, trisomy 8 and a tetraploid karyotype 92xxyy5q-. No cytotoxic treatment should be given during the preleukemic phase until transformation to acute leukemia occurs. Since preleukemic patients are very susceptible to infections, early diagnosis of the condition is important, as is supportive care in the case of surgery.
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PMID:Preleukemic syndrome in elderly patients--report of 11 cases. 385 73

A balanced translocation t(8;9) (p11;q34) was present in the peripheral blood, bone marrow, and spleen cells of a patient with Ph negative chronic myeloid leukaemia. Subsequent transformation into acute leukaemia was associated with the emergence of trisomy 8 and der(8)(8qter----cen----8p11::9q34----9qter). This is the third reported case of t(8;9) (p11;q34) and raises the question of the role of c-abl in the pathogenesis of this myeloproliferative disorder.
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PMID:Reciprocal translocation between chromosomes 8 and 9 in atypical chronic myeloid leukaemia. 386 76

Banded chromosomes of leukemic cells were studied in 53 children with chronic myeloid leukemia (CML). Ph1 chromosome was found in 21 children, and the remaining 32 cases were Ph1 negative. Besides Ph1 translocation additional chromosomal abnormalities, including marker i(17q), were revealed in three of eight children studied in blastic crisis of Ph1 positive CML. Leukemic cells of most patients with Ph1 negative CML possessed normal karyotype. Clones with chromosomal abnormalities were found in 12 of 32 cases. Most characteristic were monosomy 7 (in four children) and trisomy 8 (in three). Abnormal karyotype may be a bad prognostic sign in Ph1 negative CML. The presented data confirm the difference in age of appearance, bone marrow pattern and clinical course between Ph1 positive ("adult") and Ph1 negative (juvenile) types of CML in children. Probable prenatal commencement of CML in babies and children in the first years of life is discussed.
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PMID:Chromosome abnormalities in chronic myeloid leukemia in children. 657 82

In an attempt to relate karyotype evolution to clinical and hematological data serial chromosomal analyses were performed in 31 patients with chronic myeloid leukemia (CML), both in chronic and acute phases. Our results in Philadelphia chromosome (Ph1)-positive CML are in line with karyotype profiles described in the literature. In addition, we report on chromosomal findings in 4 cases of Ph1-negative disease, one presenting with an iso17q chromosome in the positive CML. The same chromosomal abnormality was observed in a small population of Ph1-negative cells present in one of two patients with mixed Ph1-positive/Ph1-negative CML. The first case of a female patient with the loss of a sex chromosome in Ph1-positive cells is reported. Two patients with unusually long and mild chronic phases despite the presence of trisomy 8 in their karyotypes are described. Our findings suggest that the order of appearance of additional chromosomal changes of CML is of prognostic significance for the progression and the clinical picture of the disease.
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PMID:Investigations on karyotype evolution in patients with chronic myeloid leukemia (CML). 658 Sep 30

The chromosomal involvement in the development of malignancy in chronic myeloid leukemia is not a random event. A second Ph1, a trisomy 8, an isochromosome 17q, a trisomy 17 are the main abnormalities. These aberrations use to occur as a karyotypic evolution, either simple or complicated. An extra-medullary development of blastic transformation was demonstrated by chromosomal analysis. It is difficult to demonstrate a correlation between chromosomal abnormalities and clinical evolution in the acute phase of chronic myeloid leukemia.
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PMID:[Chromosomal abnormalities of the blastic phase of chronic myeloid leukemia]. 676 78

To detect karyotype changes during the chronic phase of CML, 31 mostly Ph1-positive patients were followed cytogenetically over a period of 3 years. Newly emerging abnormalities, e.g. second Ph1-chromosome, iso17q, trisomy 8, could be correlated with clinical and hematological characteristics of the course of the disease if they constituted the first additional chromosomal change. In 54 patients with suspected or established blast crisis, TdT determinations were performed. Out of 27 patients with verified blast crisis, 6 showed markedly elevated enzyme activities in the leukemic blast cells. These patients proved to be very sensitive to cytostatic therapy with prednisone and vincristine. The continuous observation of TdT levels enabled us to initiate a controlled and, with respect to the quality of life, a less toxic therapy. Our data indicate that serial chromosomal analyses and TdT determinations are of great value in predicting prognosis and therapeutic responsiveness in CML.
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PMID:[Chromosome analysis and TdT determination: important prognostic parameters of blastic crises in chronic myelocytic leukemia]. 676 May 60


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