Rearrangement of the bcr Gene in Philadelphia-Chromosome-Negative Chronic Myeloid Leukemia

Ganesan, T. S.; Rassool, Feyruz V.; Guo, AP; Th'ng, KH; Dowding, C; Hibbin, JA; Young, BD; White, Hillary D.; Kumaran, TO; Galton, D. J.

doi:10.1007/978-3-642-72624-8_33

Cited by 51 publications

(23 citation statements)

References 15 publications

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“…The five exceptions with a BCR breakpoint were all reported by the same research group [41,42]. It would be interesting to reexamine the differential count and other clinical data to check if these patients really belong to the group of aCML or resemble more CML.…”

Section: Molecular Investigations In Ph-negative CMLmentioning

confidence: 88%

“…4) The percentage of Ph-negative CML patients with BCR rearrangement versus no BCR rearrangement varied between the different authors, e.g., Bartram et al [32,58] reported 3 out of 12 cases BCR-positive; Ganesan et al [42] and Dreazen et al 1411, 5 out of 5; Fitzgerald and Morris [46,59], 2 out of 2; WiedeInann et al [31], 8 out of 8 (5 out of 9 among the cases that were not morphologically reexamined); Kantarjian et al [60], 11 out of 23; and our group, 5 out of 12 [45, this report]. In our opinion, there are two main reasons responsible for these differences.…”

Section: Molecular Investigations In Ph-negative CMLmentioning

confidence: 99%

“…First, the different authors used clinical, hematologic and morphologic criteria that are not exactly the same, resulting in differences in diagnosis. Second, some authors [41,42] diagnosed BCR breakpoints on extra bands in only one out of several different restriction enzyme digests. In such cases, the occurrem:e of a restriction enzyme polynlorphism is a inore likely cause f()r the aberrant fragment than the l)reseo(:e of a BCR breakpoint.…”

Section: Molecular Investigations In Ph-negative CMLmentioning

confidence: 99%

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Review of clinical, cytogenetic, and molecular aspects of Ph-negative CML

Plas

Grosveld

Hagemeijer

1991

Cancer Genetics and Cytogenetics

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Section: Molecular Investigations In Ph-negative CMLmentioning

confidence: 88%

Section: Molecular Investigations In Ph-negative CMLmentioning

confidence: 99%

Section: Molecular Investigations In Ph-negative CMLmentioning

confidence: 99%

See 1 more Smart Citation

Review of clinical, cytogenetic, and molecular aspects of Ph-negative CML

Plas

Grosveld

Hagemeijer

1991

Cancer Genetics and Cytogenetics

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“…This gene is transcribed into a chimeric RNA and into a fusion bcrlabf protein. It is usually possible to detect a rearrangement in bcr, even in cases of CML that lack the typical t(9;22) translocation [38][39][40][41]. A similar rearrangement is detected in some cases of ALL and AML with the Ph' chromosome and the t(9;22) translocation.…”

Section: Dnamentioning

confidence: 99%

“…Several researchers, ourselves included, have indentified a novel 8 to [39,40], and in Ph' positive ALL with rearrangement of the bcr gene [42][43][44]. Abnormal c-abl expression has been reported in a B cell line derived from a patient with pre-B cell ALL [19].…”

Section: Rnamentioning

confidence: 99%

Oncogenes and human leukemias

Butturini¹,

Gale

1988

The International Journal of Cell Cloning

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Abstract. Eukaryotic cells contain a family of genes termed "cellular oncogenes" or "proto-oncogenes," thought to regulate normal cell growth and development. In some circumstances, such as following transduction by retroviruses, activation of these genes causes tumors and leukemias in animals. Possible mechanisms of cellular oncogene activation include: 1) DNA point mutation, deletion or insertion, 2) gene amplification, 3) gene activation by internal rearrangement, chromosomal translocation or promoter insertion, 4) recombinative events resulting in the formation of novel chimeric genes, and others.

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Characteristics and outcome of patients with Philadelphia chromosome negative,bcr/ablnegative chronic myelogenous leukemia

Onida

Ball

Kantarjian

et al. 2002

Cancer

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BACKGROUNDUp to 5% of patients with chronic myelogenous leukemia (CML) do not have the Philadelphia (Ph) translocation t(9;22)(q34;q11) or a bcr/abl molecular rearrangement. Although the diagnostic criteria of this entity are still under debate, there is general agreement that patients with Ph negative, bcr/abl negative CML have a severe clinical course that is not affected significantly by current treatment options.METHODSA population of 76 patients with bcr/abl negative CML who had received minimal or no previous therapy was characterized carefully with the intent of investigating clinical and hematologic variables and their association with survival by univariate, correlation, and multivariate analyses. A group of 73 patients with Ph negative CML who were not tested for the bcr/abl rearrangement (bcr/abl unknown) was analyzed separately and used for extension of the analysis.RESULTSIn the bcr/abl negative patient population, the median overall survival was 24 months. At the time of the analysis, 38 patients (50%) had died, and blastic transformation preceded death in 31%. Chromosomal abnormalities were found in 30% of the 76 patients, with trisomy 8 the most common abnormality. Complex chromosomal abnormalities were rare, and monosomy 7 was not observed. Survival was not affected significantly by treatment. Multivariate analysis identified older age (> 65 years), anemia (hemoglobin < 10 g/dL), and severe leukocytosis (white blood cells > 50 × 109/L) as variables with independent prognostic significance for poor survival. A prognostic scoring system stratified patients into a low‐risk group (53%) and a high‐risk group (47%), with median survivals of 38 months and 9 months, respectively.CONCLUSIONSBcr/abl negative CML is a distinct clinical entity associated with very poor prognosis. Two risk categories are identifiable using a simple scoring system based on age, hemoglobin level, and leukocyte number. Cancer 2002;95:1673–84. © 2002 American Cancer Society.DOI 10.1002/cncr.10832

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Rearrangement of the bcr Gene in Philadelphia-Chromosome-Negative Chronic Myeloid Leukemia

Cited by 51 publications

References 15 publications

Review of clinical, cytogenetic, and molecular aspects of Ph-negative CML

Review of clinical, cytogenetic, and molecular aspects of Ph-negative CML

Oncogenes and human leukemias

Characteristics and outcome of patients with Philadelphia chromosome negative,bcr/ablnegative chronic myelogenous leukemia

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