title: Philadelphia chromosome positive childhood acute lymphoblastic leukemia: clinical and cytogenetic characteristics and treatment outcome. A Pediatric Oncology Group study.
abstract: Among 3,638 children with acute lymphoblastic leukemia (ALL) entered on Pediatric Oncology Group (POG) protocols between June 1981 and April 1989, successful cytogenetic studies were available for 2,519, 58 (2.3%) of which had the Philadelphia (Ph) chromosome detected. Features associated with the presence of the Ph chromosome were high leukocyte count (median, 33 x 10(9)/L), older age median, 9.6 years), a higher proportion of French-American-British L2 morphology, and a lower frequency of mediastinal mass. Immunologic marker studies at diagnosis in 56 Ph+ cases identified early pre-B ALL in 42 cases (75%), pre-B-cell in 9 (16%), and T-cell in 5 (9%). This distribution is similar to that found in Ph+ ALL. Intensive multiagent chemotherapy induced complete remissions in only 78% of eligible Ph+ patients compared with 96% of those without an identified Ph chromosome (P less than .001). Of 44 eligible Ph+ patients treated on POG frontline protocols for children with non-T, non-B-cell ALL, 27 have failed therapy, compared with 520 of 1,892 without an identified Ph chromosome (logrank P less than .001). Ph+ ALL is an aggressive form of acute leukemia that frequently presents in older children with a high leukocyte count, FAB L2 morphology, and a pseudodiploid karyotype, and becomes multidrug-resistant early. Thus, Ph+ cases require early identification to permit treatment with intensive induction regimens and experimental approaches such as bone marrow transplantation.
authors.affiliation: St Jude Children's Research Hospital.
authors.forename: W
authors.initials: W
authors.lastname: Crist
authors.forename: A
authors.initials: A
authors.lastname: Carroll
authors.forename: J
authors.initials: J
authors.lastname: Shuster
authors.forename: J
authors.initials: J
authors.lastname: Jackson
authors.forename: D
authors.initials: D
authors.lastname: Head
authors.forename: M
authors.initials: M
authors.lastname: Borowitz
authors.forename: F
authors.initials: F
authors.lastname: Behm
authors.forename: M
authors.initials: M
authors.lastname: Link
authors.forename: P
authors.initials: P
authors.lastname: Steuber
authors.forename: A
authors.initials: A
authors.lastname: Ragab
mesh_terms: D001215:Asparaginase / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D007141:Immunoglobulin Fc Fragments / Q000235:genetics / Q000276:immunology; D008297:Male; D010641:Phenotype; D010677:Philadelphia Chromosome; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use; D011379:Prognosis; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D007141:Immunoglobulin Fc Fragments; D014750:Vincristine; D001215:Asparaginase; D011241:Prednisone