Cancer Institute A national cancer institute
designated cancer center

Michael Link

Publication Details

  • PHILADELPHIA-CHROMOSOME AND MONOSOMY-7 IN CHILDHOOD ACUTE LYMPHOBLASTIC-LEUKEMIA - A PEDIATRIC ONCOLOGY GROUP-STUDY BLOOD Russo, C., Carroll, A., Kohler, S., Borowitz, M., Amylon, M., Homans, A., Kedar, A., Shuster, J., Land, V., Crist, W., Pullen, J., Link, M. 1991; 77 (5): 1050-1056

    Abstract:

    During an 8-year period, 3,638 children from institutions of the Pediatric Oncology Group (POG) were diagnosed with acute lymphoblastic leukemia (ALL). Fifty-seven patients had Philadelphia chromosome-positive (Ph1) ALL. Blast cells obtained at diagnosis from 13 of these 57 cases (23%) were also found to have partial or complete monosomy 7 (-7). This subgroup of children with Ph1/-7 ALL was comprised primarily of older males with early B-lineage ALL. Bone marrow specimens from six Ph1/-7 patients were studied further using the polymerase chain reaction and primers that flank the ALL, and chronic myelogenous leukemia breakpoints to determine the molecular characteristic of the 9;22 translocation. Rearrangements were detected in RNA from bone marrow and/or peripheral blood cells of six patients, although four were in hematologic remission at the time of the analysis. Five cases showed the ALL breakpoint, while one child with Ph1/-7 showed the chronic myelogenous leukemia breakpoint. The induction failure rate was much higher in this subgroup (31%) as compared with Ph1-negative cases, and the projected duration of event-free survival reflected the aggressive nature of this subgroup because no children are projected to remain in remission at 2 years. ALL with both the 9;22 translocation and -7 appears to represent a unique and previously undescribed subgroup of childhood ALL associated with a particularly adverse outcome. Leukemic transformation in such patients may involve the interaction of a dominant oncogene (Ph1) and a tumor suppressor gene (-7).

    View details for Web of Science ID A1991EZ33000019

    View details for PubMedID 1995090

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