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)

A child with Ph1-negative juvenile chronic myelogenous leukemia (CML) is presented. The only chromosomal abnormality in hematopoietic tissues consisted of an absent Y chromosome. While a missing Y chromosome in adult patients with CML may be associated with a better prognosis, the clinical course in our patient was as malignant as that usually observed in other children with Ph1-negative juvenile CML.
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PMID:Missing Y chromosome in juvenile chronic myelogenous leukemia. 105 71

Juvenile chronic myeloid leukemia (JCML) is an unusual subtype of children's leukemia, characterized by unique clinical presentation. Recent studies revealed several biological features, distinguishing from those observed in adult type chronic myeloid leukemia (ACML). The majority of ACML cases are characterized by the presence of an hybrid bcr-abl rearranged gene. In an effort to elucidate the molecular basis of this unusual leukemia we looked for bcr rearrangement in six JCML cases. No bcr rearrangement was identified in any of the analyzed samples. Together with previous studies from JCML cases, JCML has a different mechanism of leukomogenesis from ACML.
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PMID:Lack of bcr rearrangement in juvenile chronic myeloid leukemia. 168 80

We performed cytogenetic studies and breakpoint cluster region (bcr) rearrangement analysis in two cases of juvenile chronic myeloid leukemia (JCML) which is special type of chronic myeloid leukemia (CML). Case 1 (8-month-old male) and case 2 (3-month-old female) showed clinical and hematologic manifestations similar to CML. Each of case 1 and 2 had normal karyotype and no bcr rearrangement. These findings suggest that JCML is a different heterogeneous disorder from that of adult CML.
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PMID:[No rearrangement of the breakpoint cluster region in two juvenile chronic myeloid leukemia]. 189 Jul 47

Two spontaneous outgrowing Epstein-Barr virus (EBV)-carrying lymphoblastoid cell lines (LCLs), CG2 and CG3, have been established from bone marrow cells of myeloid leukemia patients. CG2 was derived from a patient with chronic myelomonocytic leukemia (CMMoL) and who has a 45 XO karyotype. CG3 was derived from a patient with juvenile chronic myeloid leukemia (CML) and who carries a hypotetraploid karyotype, 91XXYY. Both CG2 and CG3 cells carry the same type of translocation; t(1;19)(q23;p13). Both cell lines are of an early B cell lineage as shown by their reactivities with monoclonal antibodies OKIa, B1, B2 and B4. The combination of horizontal discontinuous agarose slab gel and Southern hybridization results show CG2 and CG3 cells are of monoclonal origin and harbor episomal EBV genomes. Approximately 50 EBV genome equivalents were contained in CG2 and CG3 cells. Immunofluorescence studies demonstrate the expression of EBV-encoded antigen (EBNA) in almost all cells of these two lines. The expression of EA and VCA is only observed in a small percentage of cells and cannot be induced by treatment with TPA and SB. Therefore, CG2 and CG3 cells are probably nonproducer cell lines for EBV. The serum samples from both patients have been shown to contain elevated IgG antibody titers to EBV antigens. Both cells are found to be nontumorigenic in nude mice. These cells may provide an important tool in analyzing molecular epidemiological aspects of EBV infections in diseases such as CMMoL and juvenile CML.
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PMID:Characterization of two newly established EBV-containing lymphoblastoid cell lines from patients with myeloid leukemias. 215 89

Bone marrow transplantation plays an essential role in the successful treatment of both juvenile and adult chronic myelogenous leukemia. Recently, it has been reported that conditioning with high doses of busulfan can successfully replace total body irradiation (TBI), in patients with acute myelogenous leukemia as well as adult chronic myelogenous leukemia. We report here the case of a 29-month-old boy with juvenile chronic myelogenous leukemia (JCML) transplanted with HLA-identical bone marrow after conditioning with busulfan, etoposide and cyclophosphamide. Successful engraftment was followed by early relapse on day 67. A second HLA-identical transplant was performed following myeloablative treatment with TBI. Engraftment was once again successful and the patient remains free of disease more than 24 months after transplantation. We conclude that busulfan is insufficient in eradicating JCML and that TBI is required prior to transplantation.
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PMID:Busulfan/cyclophosphamide plus bone marrow transplantation is not sufficient to eradicate the malignant clone in juvenile chronic myelogenous leukemia. 219 Jun 61

A case of a boy with juvenile chronic myelogenous leukemia (CML) and independent clonal abnormalities of chromosomes 4 and 5 is presented. The characteristics and cytogenetics of CML are discussed, as is the involvement of chromosomes 4 and 5 in hematologic malignancies. The significance of these karyotypic findings in juvenile CML is explored.
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PMID:Juvenile chronic myelogenous leukemia with abnormalities of chromosomes 4 and 5. 229 80

BMT is a well-established treatment for children with ALL in second remission, ANLL in first and second remission and children with JCML and CML. Improvements in transplantation technology and supportive care have resulted in significant increases in the percentage of long-term survivors of allogeneic marrow transplantation. Newer strategies, such as partially matched donor, unrelated matched donor, and autologous transplants, are bineg pursued to overcome the histocompatability barrier. The development of more effective antileukemic cytoreductive chemotherapy and radiation therapy regimens and better methods of preventing GVHD are areas in which further improvements are necessary. Newer methods of marrow purging, such as the use of monoclonal antibodies linked to immunotoxins, already are being tested. In addition, the recent development of molecularly cloned hematopoietic growth factors, such as CSFGM, may make it possible to improve marrow recovery and hasten return of normal immunologic function, thereby increasing the overall safety of the transplant procedure. It is hoped that these innovations eventually will increase the overall applicability of BMT and its role in the treatment of leukemia.
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PMID:Bone marrow transplantation for treatment of leukemia in children. 304 59

Juvenile chronic myelogenous leukemia (JCML) is a malignant hematopoietic disorder of monocyte-histiocyte lineage that affects children less than 4 years of age. Since the disease represents only 2% of all childhood leukemias, experience with it has been limited even in large centers. This review summarizes our 10 year institutional study of JCML as well as a comprehensive literature survey. The goal of the article is to underscore the cardinal features of juvenile chronic myelogenous leukemia that are useful for diagnosis, and to highlight recent advances in the understanding of the biology and treatment of the disease.
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PMID:Juvenile chronic myelogenous leukemia. 305 47

Using cell culture studies specific in-vitro characteristics have been reported for Philadelphia chromosome positive myelogenous leukemia (Ph+ CML) and for juvenile chronic myelogenous leukemia (JCML) previously. We performed cell culture studies in four patients with chronic myelomonocytic leukemia (CMML) and demonstrated the following in-vitro features: excessively increased circulating CFU-C, while BFU-E and CFU-mix were either moderately increased or not detectable; CFU-C colony formation from CMML mononuclear cells (MNC) without addition of exogenous colony stimulating activity (CSA), even after depletion from adherent cells; failing inhibition of CMML MNC on normal BFU-E colony formation. These in-vitro characteristics point to CMML as a distinct entity. In two CMML-patients investigated CFU-C proliferation appeared to some extent inhibited by the addition of IFN-alpha, IFN-gamma and TNF-alpha to cell cultures.
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PMID:Colony growth characteristics in chronic myelomonocytic leukemia. 316 85

To characterize juvenile chronic myelogenous leukemia (JCML), the proliferative properties of bone marrow (BM) and peripheral blood (PB) cells from nine patients were studied using assays for CFU-C and CFU-GEMM and liquid cultures. All specimens showed two reproducible abnormalities: impaired growth of normal hematopoietic progenitors and excessive proliferation of monocyte-macrophage colonies in the absence of exogenous colony-stimulating activity (CSA). Cytogenetic studies in one patient indicated that the CFU-C were malignant because BM cells at diagnosis and monocyte-macrophage colonies showed an abnormal karyotype, whereas PB lymphocytes did not. In contrast to JCML, PB from six adults with Philadelphia (Ph1) chromosome-positive chronic myelogenous leukemia (Ph1 + CML) yielded CSA-dependent CFU-C colonies which were composed of granulocytes, macrophages, or both, as well as exuberant growth of BFU-E colonies. Co-cultures of JCML BM adherent or nonadherent cells with normal BM resulted in suppression of normal hematopoietic colony formation. Supernatant from JCML adherent cells in liquid culture or plasma from newly diagnosed untreated JCML patients also suppressed control BM colony growth in a dose-dependent manner. These findings confirm that JCML is a malignant disorder of monocytic lineage and suggest that the mechanism of hematopoietic failure in JCML is mediated by an inhibitory monokine secreted by malignant JCML cells.
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PMID:Juvenile chronic myelogenous leukemia: characterization of the disease using cell cultures. 348 10


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