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Philadelphia chromosome positive childhood acute lymphoblastic leukemia:
clinical and cytogenetic characteristics and treatment outcome. A Pediatric
Oncology Group study
W Crist, A Carroll, J Shuster, J Jackson, D Head, M Borowitz, F Behm, M Link, P Steuber and A Ragab
St Jude Children's Research Hospital.
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.
Volume 76,
Issue 3,
pp. 489-494,
08/01/1990
Copyright © 1990 by The American Society of Hematology
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