Prognostic Factors in Philadelphia+/BCR-ABL+ Acute Lymphoblastic Leukemia

Abstract

Adult patients with acute lymphoblastic leukemia (ALL) expressing the t(9;22)/Philadelphia chromosome (Ph+) or the corresponding BCR-ABL rearrangements (BCR+, P190 and P210) constitute the prognostically worst ALL subtype [1,2]. Although complete remission (CR) rates are 56%-96%, the median duration of CR and survival is one year or less and <20% of patients survive disease-free for >2 years. Disease-free survival can be improved by allogeneic bone marrow transplantation, while the role of high-dose treatments supported by autograft is presently less well defined [3-7]. However Ph/BCR+ ALL behaves heterogeneously, and some patients may benefit from chemotherapy alone. Longer remissions have been reported in cases with hyperdiploid karyotype and in other selected subgroups [8-10]. We noted that, using Anthracycline-based consolidation, outcome was improved in a fraction of cases [11]. In another study, some patients who converted to BCR negative status after intensive treatment without allogeneic transplantation were in remission for >3 years [12]. Prolonged remissions were also described in subsets of childhood Ph/BCR+ ALL treated with aggressive modern-type chemotherapy regimens [13,14]. In the light of these reports, we review the long-term outcome of an unselected patient population of 76adults with Ph/BCR+ ALL, treated over a 25-year period with regimens developed by the L-B-V Group (St. Bartholomew’s Hospital, London, UK; Ospedali Riuniti, Bergamo, Italy; Ospedale Civile, Vicenza, Italy).