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Philadelphia chromosome and monosomy 7 in childhood acute lymphoblastic leukemia: a Pediatric Oncology Group study
| Content Provider | Scilit |
|---|---|
| Author | Russo, C. Carroll, A. Kohler, S. Borowitz, M. Amylon, M. Homans, A. Kedar, A. Shuster, J. Land, V. Crist, W. |
| Copyright Year | 1991 |
| Description | 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). |
| Related Links | https://ashpublications.org/blood/article-pdf/77/5/1050/221566/1050.pdf |
| Ending Page | 1056 |
| Page Count | 7 |
| Starting Page | 1050 |
| DOI | 10.1182/blood.v77.5.1050.bloodjournal7751050 |
| Journal | Blood |
| Issue Number | 5 |
| Volume Number | 77 |
| Language | English |
| Publisher | American Society of Hematology |
| Publisher Date | 1991-03-01 |
| Access Restriction | Open |
| Subject Keyword | Pediatrics and Child Health Children Survival Bone Marrow Pediatric Oncology Chromosome Ph1 Philadelphia Lymphoblastic Journal: Blood (Vol- 82, Issue- 5) |
| Content Type | Text |
| Resource Type | Article |