title: Pretreatment characteristics and clinical outcome of acute promyelocytic leukaemia patients according to the PML-RAR alpha isoforms: a study of the PETHEMA group.
abstract: Of 167 newly diagnosed acute promyelocytic leukaemia patients, 83 patients were long (L)-form (50%), eight variable (V)-form (5%) and 76 short (S)-form (45%). The V-form and S-form groups presented a significantly higher percentage of patients with white blood cell counts > 10 x 10(9)/l (P < 0.05). The S-form cases displayed a significantly higher number of cases with M3v microgranular features (P = 0.005) and CD34 expression (P < 0.0001). There were no differences between the three isoforms in complete remission (CR) rate (overall CR 90%), but the 3-year disease-free survival was lower for V-form cases than it was for L- and S-form cases (62% vs. 94% and 89%, P = 0.056). We conclude that the V-form and S-form types are associated with some negative prognostic features at diagnosis. However, our data were only able to demonstrate an association with adverse prognosis in the V-form type and, moreover, as the number of cases was limited, needs to be confirmed in large, uniformly treated series.
authors.affiliation: Department of Haematology, Hospital Universitario, Salamanca, Spain. margondi@usal.es
authors.forename: M
authors.initials: M
authors.lastname: González
authors.forename: E
authors.initials: E
authors.lastname: Barragán
authors.forename: P
authors.initials: P
authors.lastname: Bolufer
authors.forename: C
authors.initials: C
authors.lastname: Chillón
authors.forename: D
authors.initials: D
authors.lastname: Colomer
authors.forename: R
authors.initials: R
authors.lastname: Borstein
authors.forename: M J
authors.initials: MJ
authors.lastname: Calasanz
authors.forename: M T
authors.initials: MT
authors.lastname: Gómez-Casares
authors.forename: A
authors.initials: A
authors.lastname: Villegas
authors.forename: I
authors.initials: I
authors.lastname: Marugán
authors.forename: J
authors.initials: J
authors.lastname: Román
authors.forename: G
authors.initials: G
authors.lastname: Martín
authors.forename: C
authors.initials: C
authors.lastname: Rayón
authors.forename: G
authors.initials: G
authors.lastname: Debén
authors.forename: M
authors.initials: M
authors.lastname: Tormo
authors.forename: J
authors.initials: J
authors.lastname: Díaz-Mediavilla
authors.forename: J
authors.initials: J
authors.lastname: Esteve
authors.forename: J
authors.initials: J
authors.lastname: González-San Miguel
authors.forename: C
authors.initials: C
authors.lastname: Rivas
authors.forename: K
authors.initials: K
authors.lastname: Pérez-Equiza
authors.forename: R
authors.initials: R
authors.lastname: García-Sanz
authors.forename: F J
authors.initials: FJ
authors.lastname: Capote
authors.forename: J M
authors.initials: JM
authors.lastname: Ribera
authors.forename: J
authors.initials: J
authors.lastname: Arias
authors.forename: A
authors.initials: A
authors.lastname: León
authors.forename: M A
authors.initials: MA
authors.lastname: Sanz
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D018952:Antigens, CD34 / Q000032:analysis; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D015473:Leukemia, Promyelocytic, Acute / Q000188:drug therapy / Q000276:immunology / Q000378:metabolism*; D007958:Leukocyte Count; D008297:Male; D008875:Middle Aged; D009363:Neoplasm Proteins / Q000235:genetics*; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D016133:Polymerase Chain Reaction / Q000379:methods; D011379:Prognosis; D016016:Proportional Hazards Models; D020033:Protein Isoforms / Q000235:genetics; D016896:Treatment Outcome; D014212:Tretinoin / Q000627:therapeutic use
chemical_list: D018952:Antigens, CD34; D009363:Neoplasm Proteins; D015514:Oncogene Proteins, Fusion; D020033:Protein Isoforms; C093476:promyelocytic leukemia-retinoic acid receptor alpha fusion oncoprotein; D014212:Tretinoin