title: Clinical sensitivity to anthracyclines in PH/BCR+ acute lymphoblastic leukemia.
abstract: Translocation t(9;22) or Philadelphia chromosome (Ph)/BCR-ABL rearrangement positive acute lymphoblastic leukemia (Ph/BCR+ ALL) is associated with a very short survival of about one year in most patients. We analyzed long-term outcome of 76 adults with Ph/BCR+ ALL, in order to detect which factors were associated with longer survival. Modifiable prognostic factors included type of treatment, allogeneic marrow transplant (allo-BMT), and early anthracycline dose intensity (high = H/A, low = L/A); unmodifiable factors were age, gender, FAB morphology, phenotype, blast count, P190/210 transcript, hepatospleno-lymphadenopathy, LDH level. Median patient age was 43 years (range 15-71). Four favorable prognostic factors (FPF) were found associated with greater likelihood of complete remission (blast count < 50 x 10(9)/l, p = 0.08), longer remission duration (age < 50 years, p < 0.001; H/A, p < 0.05), and lower relapse rate (allo-BMT, p = 0.017). Age and anthracycline dose intensity exerted a synergistic prognostic effect. According to the cumulative incidence of FPF in each patient (FPF 0-1 = 29, 2-3 = 42, 4 = 5), the probability of survival increased from nil to 0.22 to 0.60 at 5 years (p < 0.005). Adult Ph/BCR+ ALL is relatively sensitive to anthracyclines, which therefore should be prescribed at full dosage to patients not eligible to allo-BMT or in the waiting list for unrelated donor transplantation.
authors.affiliation: Division of Hematology, Ospedali Riuniti, Bergamo, Italy.
authors.forename: R
authors.initials: R
authors.lastname: Bassan
authors.forename: A Z
authors.initials: AZ
authors.lastname: Rohatiner
authors.forename: A
authors.initials: A
authors.lastname: Rambaldi
authors.forename: T
authors.initials: T
authors.lastname: Lerede
authors.forename: E
authors.initials: E
authors.lastname: Di Bona
authors.forename: M
authors.initials: M
authors.lastname: Carter
authors.forename: G
authors.initials: G
authors.lastname: Rossi
authors.forename: E
authors.initials: E
authors.lastname: Pogliani
authors.forename: G
authors.initials: G
authors.lastname: Lambertenghi-Deliliers
authors.forename: P
authors.initials: P
authors.lastname: Fabris
authors.forename: A
authors.initials: A
authors.lastname: Porcellini
authors.forename: T A
authors.initials: TA
authors.lastname: Lister
authors.forename: T
authors.initials: T
authors.lastname: Barbui
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001752:Blast Crisis / Q000276:immunology / Q000473:pathology; D002892:Chromosomes, Human, Pair 22; D002899:Chromosomes, Human, Pair 9; D005260:Female; D005500:Follow-Up Studies; D016044:Fusion Proteins, bcr-abl / Q000235:genetics; D015321:Gene Rearrangement; D006801:Humans; D016130:Immunophenotyping; D008297:Male; D008875:Middle Aged; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy* / Q000235:genetics* / Q000401:mortality / Q000473:pathology; D011379:Prognosis; D015996:Survival Rate; D013997:Time Factors; D014178:Translocation, Genetic
chemical_list: D016044:Fusion Proteins, bcr-abl