PHILADELPHIA-CHROMOSOME-POSITIVE ADULT ACUTE LYMPHOBLASTIC-LEUKEMIA -CHARACTERISTICS, PROGNOSTIC FACTORS AND TREATMENT OUTCOME

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.