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)

Myeloproliferative disease of childhood is frequently associated with chromosomal anomalies, usually of the C group. Clinical features are similar to those of the juvenile type of chronic myeloid leukemia. A child with this disease is described. Marked myeloid proliferation, anemia, thrombocytopenia and hepatosplenomegaly were present; leukocyte alkaline phosphatase and fetal hemoglobin were moderately elevated. Chromosome analysis of bone marrow cells revealed a mosaicism 47,XX,+21/46,XX. Down's syndrome was ruled out by the child's normal phenotype and dermatoglyphic analysis. The cytogenetic finding is probably evidence for the clonal origin of the trisomy 21 cell line.
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PMID:Myeloproliferative disease of childhood associated with a trisomy 21 clone. 11 7

Fetal and adult erythrocyte characteristics were studied serially in a 30-mo-old female with juvenile chronic myelocytic leukemia. On presentation the erythrocytes exhibited predominantly fetal characteristics as indicated by 69% hemoglobin F (HbF), 1.1% hemoglobin A2 (HbA2), absent I antigen, and fetal levels of the erythrocyte enzymes, carbonic anhydrase I and II, glucose-6-phosphate dehydrogenase, hexokinase, pyruvate kinase, and lactate dehydrogenase; 100% of the erythrocytes present contained HbF. However, Orskov-Jacobs-Stewart hemolysis demonstrated that at least one adult characteristic was present. Seven months later HbF was 17%; I antigen and carbonic anhydrase I had increased to adult levels. The number of cells containing HbF had decreased to 30%. Further studies indicated that at least three new populations of red cells were present after 7 mo which had not previously been detected. Two of these populations exhibited a mixture of both fetal and adult characteristics. Such findings suggested that an ongoing disturbance of regulatory mechanisms was responsible for the variable expression of fetal versus adult erythrocyte characteristics.
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PMID:Changing erythrocyte populations in juvenile chronic myelocytic leukemia: evidence for disordered regulation. 26 91

Because all erythroid cells in chronic myelogenous leukemia are believed to arise from a common pluripotent stem cell, we studied the cellular distribution of fetal hemoglobin in patients with the disease to test whether cells with and without hemoglobin F have a common or separate pluripotent stem-cell origin. In a patient with chronic myelogenous leukemia and sickle/beta thalassemia, 17 per cent of the red cells during remission and 18 to 22 per cent during the blastic phase contained fetal hemoglobin. In 24 non-hemoglobinopathic leukemic patients 0.3 to 28 per cent of red cells contained hemoglobin F. Since the erythrocytes in chronic myelogenous leukemia are almost exclusively derived from the malignant clone, these data suggest that cells containing fetal hemoglobin originate from the same pluripotent stem-cell progenitor as those without hemoglobin F. The findings argue against a separate line of "partially switched" pluripotent stem cells as being responsible for maintenance of hemoglobin F production in the adult.
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PMID:Cellular distribution of hemoglobin F in a clonal hemopoietic stem-cell disorder. 27 Dec 71

Between 1959 and 1973 were analyzed the records of 798 patients with chronic myelocytic leukemia. Mean survival (MS) for the entire group is 42 months. 342 patients have been followed closely during and after development of blastic transformation. Presence of following symptoms at the time of diagnosis: asthenia, weight loss, bone pain, fever, sweats and digestive disorders is of poor prognosis significance (MS: 36 months, no sign: MS 75 months) (P less than 0.001). Spleen size is also a prognostic factor. MS are respectively 70, 52 and 35 months if initial splenomegaly is moderate (less than 3 cm), marked (less than 6 cm) or tumoral (greater than or equal to 6 cm). Thrombocytopenia (less than 15,000/mm3 or thrombocythemia (greater than 1 million/mm3) have a poor prognosis with median survival 22 months and 28 months. If peripheral blast cells (hemocytoblasts + myeloblasts) exceed 5%, the prognosis is worse; beyond 10% MS is 26 months. In contrast certain factors have better prognosis: hemoglobin greater than or equal to 14 g/100 ml, young age (less than 20 y.) MS: 62 months), female sex and an initial WBC count below 25 x 10(3)/mm3 (MS: 70 months).
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PMID:Prognostic factors in chronic granulocytic leukemia. A study of 798 cases. 28 95

Isolated comma or corkscrew-shaped venular segments in the conjunctiva have been felt to be diagnostic of sickle cell disease. External eye examination of a 34-year-old black man with a two-year history of chronic granulocytic leukemia revealed multiple small and medium-sized isolated conjunctival venular segments. Laboratory findings at the time of examination included a white blood cell count of 132,000/mm3 with 87% eosinophils, a hematocrit reading of 30%, and hemoglobin AA. Since whole blood hyperviscosity can be present in chronic granulocytic leukemia as well as sickle cell disease, this may account for the similarly of the conjunctival changes.
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PMID:Comma-shaped venular segments of conjunctiva in chronic granulocytic leukemia. 105 96

The chromosome constitution of myeloid cells of 106 patients with chronic myelocytic leukemia (CML) was studied. Two thirds of the patients were in the chronic and one third in the blastic phase. Particular attention was paid to patients with a mixture of Ph1-positive and Ph1-negative metaphases, and those with additional chromosome anomalies. A statistical analysis of the additional anomalies observed during the chronic and the blastic phase waandom involvement of certain chromosome groups. Chromosomal findings in medullary and extramedullary tissues were compared; the results suggest an accumulation of cells with additional anomalies in extramedullary sites. Ph1-negative patients were shown to be older and to have a survival shorter than Ph1-positive patients. The medium survival time of patients in the blastic phase may be longer in those without additional chromosome anomalies. Apart from the chromosomal status, hemoglobin concentration, the number of platelets and the percentage of myeloblasts in the bone marrow had a definite influence on survival times.
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PMID:Chronic myelocytic leukemia: cytogenetic findings and their relations to pathogenesis and clinic. 107 Dec 84

A total of 40 evaluable patients were treated for blastic crisis of chronic myelogenous leukemia with mitoxantrone, 12 mg/m2 per day for three days and 5-azacytidine 150 mg/m2 per day for 5 days. Toxicity was primarily hematologic and was manageable. The overall response rate was 23%, including five complete responders, two partial responders, and two with hematologic improvement. Cytogenetic and immunophenotypic characterization of the leukemia was performed on all patients with aspirable bone marrow, and these results were correlated with response and survival, but did not have predictive value once the patient was in blastic crisis. Only initial platelet count (p = 0.02), hemoglobin (p = 0.03), and lower white blood cell count (p = 0.09) were somewhat predictive of response. Lack of hepatic involvement (p = 0.05), lower white blood cell count (0.05), and higher platelet count (p = 0.02) were predictive of prolonged survival. Although response did not strongly correlate with survival, one third of responders were alive at one year. This regimen produces results similar to those of other recently published regimens in this disease. Earlier intervention and more effective therapy is necessary in these patients.
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PMID:Phase II study of mitoxantrone and 5-azacytidine for accelerated and blast crisis of chronic myelogenous leukemia: a study of the Eastern Cooperative Oncology Group. 137 12

A 42-year-old male was diagnosed as having Ph-positive chronic myelogenous leukemia (CML) in 1988. He had been treated with ranimustine and interferon alpha. In April 1990, he was admitted to our hospital because of hemorrhagic diathesis. Blood counts revealed a white blood cell count of 319,200/microliters with 12 per cent blasts, a hemoglobin level of 9.2 g/dl, and a platelet count of 48,000/microliters. The bone marrow aspiration revealed hypercellularity with 68.2 per cent blasts, and chromosomal analysis showed 48, XY, +8, double Ph. A combination chemotherapy containing vindesine, cytarabine and prednisolone was administered. Four days later, he suddenly complained of headache and vertigo. CT scan of the brain showed a high density area at the cerebellar vermis. He was then treated with intensive combination chemotherapy including enocitabine, daunomycin, 6-mercaptopurine and prednisolone. He attained a hematological response and clinical improvement temporarily, as the cerebellar tumor regressed. In September he had headache and vertigo again, and CT scan revealed a rapid increase in size of the cerebellar tumor. Local irradiation with total doses of 19 Gy brought about a partial resolution of the lesion, and relief from the symptoms. In November, his hematological conditions deteriorated gradually and he died of brain hemorrhage on November 22, 1990. Post-mortem examination disclosed a 1 x 1 cm sized mass in the cerebellar vermis which showed a fibrous change surrounded with hemosiderin-laden macrophages microscopically. We reviewed the eight reported cases of CML with intracranial tumors, and discussed the factors which had contributed to the prolongation of survival in our patient.
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PMID:[Isolated cerebellar tumor formation in a patient with blastic crisis of chronic myelogenous leukemia]. 143 48

Serum erythropoietin (Epo) titers in patients with various hematological malignancies and related diseases were determined by radioimmunoassay. Serum Epo titer was inversely correlated with hemoglobin concentration in iron deficiency anemia, aplastic anemia, myelodysplastic syndromes (MDS), acute leukemia, malignant lymphoma, multiple myeloma and myelofibrosis, but there was no correlation between serum Epo titer and hemoglobin concentration in chronic myelogenous leukemia or polycythemias. Serum Epo titers in aplastic anemia were much higher than those in iron deficiency anemia. Serum Epo titers in MDS, malignant lymphoma and multiple myeloma differed considerably among patients. Serum Epo titers in untreated polycythemia vera were significantly lower than in treated polycythemia vera or secondary polycythemia.
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PMID:Serum erythropoietin titers in hematological malignancies and related diseases. 146 Mar 22

The multistep nature of human cancers is well illustrated by chronic myelogenous leukemia (CML), a clonal hematologic malignancy with two distinct phases: chronic and acute. Transition between these phases is characterized by unregulated growth and loss of differentiation of myeloid cells and their progenitors. We recently reported that loss of normal p53 expression correlates with transition from the chronic to acute phase in at least 25% of cases of CML. However, the precise relationship between this loss and biologic features of acute-phase CML is uncertain. To study this question, we artificially expressed normal p53 in K562, an erythroid acute-phase CML cell line lacking normal p53 expression. Biological effects were assessed by determining several growth parameters and by measuring synthesis of hemoglobin, a feature of mature erythroid cells. K562 cells expressing normal p53 had an increased proportion of cells in G1 versus S + G2, a longer doubling time and a lower growth saturation density than control K562 cells or K562 cells with antisense p53. Cells with normal p53 also expressed up to 50-fold more hemoglobin than controls. These data are consistent with the notion that loss of p53 expression may be responsible for many of the features of acute-phase CML cells. The data also demonstrate direct involvement of p53 in differentiation processes.
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PMID:Expression of the normal p53 gene induces differentiation of K562 cells. 150 93


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