X. Thomas et al., PHILADELPHIA-CHROMOSOME-POSITIVE ADULT ACUTE LYMPHOBLASTIC-LEUKEMIA -CHARACTERISTICS, PROGNOSTIC FACTORS AND TREATMENT OUTCOME, HEM CELL TH, 40(3), 1998, pp. 119-128
Philadelphia chromosome positive (Ph+) acute lymphoblastic leukemia (A
LL) is an aggressive form of acute leukemia that represents about one
third of all adult ALL. Between 1984 and 1996, forty-three cases of Ph
+ ALL (22 males and 21 females) were diagnosed in our institution by s
uccessful cytogenetic studies and/or molecular biology. Median age was
42 years (range, 20-71 years) with 28 patients aged below 50 years. M
edian leukocyte count was 39.7 x 10(9)/l on admission. Tumoral syndrom
e was seen only in 21 patients (49%) of which 4 cases presented with c
entral nervous system (CNS) involvement. Among the 38 patients classif
ied according to the French-American-British (FAB) criteria, 26 showed
L1 and 9 L2 morphology. Three patients showed undifferentiated leukem
ia. Immunological study at diagnosis only showed B-cell lineage ALL wi
th 95% of patients expressing CD10 and 50% expressing CD20. The Ph+ as
sole anomaly was seen in 13 patients (31%), while additional chromoso
me changes were observed in 28 cases. Two patients were diagnosed only
on molecular biology showing a Bcr/Abl rearrangement. Thirty-nine pat
ients treated according to LALA protocols were eligible for the analys
is of treatment outcome. Complete remission (CR) was achieved in 25 ca
ses (64%, 95% CI: 47-79%). The median disease-free survival (DFS) and
the median overall survival were 6 and 9 months respectively. Relapse
was observed in 16 cases (64% of patients achieving CR). Initial param
eters associated with a statistically significant worse prognosis were
''blastic'' fever, hyperuricemia, the presence of an extra Ph chromos
ome and patients whose marrow does not contain any normal mitosis (AA
cases). As post-induction therapy, 13 cases followed a chemotherapy pr
ogram (group 1) while II received early bone marrow (BM) or peripheral
stem cell (PSC) transplantation (group 2) (5 allogeneic BM transplant
ation and 6 autologous BM or PSC transplantation). One patient did not
receive any post-induction therapy. In group 1, the median DFS and ov
erall survival were of 5 and 11 months respectively, while they were o
f 9 months and not reached respectively in group 2 with a 2-year survi
val rate of 51% (95% CI: 21-83%) confirming the requirement for intens
ified therapy in Ph+ ALL.