1. Pretreatment characteristics and clinical outcome of acute promyelocytic leukaemia patients according to the PML-RAR alpha isoforms: a study of the PETHEMA group
- Author
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M, González, E, Barragán, P, Bolufer, C, Chillón, D, Colomer, R, Borstein, M J, Calasanz, M T, Gómez-Casares, A, Villegas, I, Marugán, J, Román, G, Martín, C, Rayón, G, Debén, M, Tormo, J, Díaz-Mediavilla, J, Esteve, J, González-San Miguel, C, Rivas, K, Pérez-Equiza, R, García-Sanz, F J, Capote, J M, Ribera, J, Arias, A, León, and M A, Sanz
- Subjects
Adult ,Male ,Adolescent ,Oncogene Proteins, Fusion ,Infant, Newborn ,Infant ,Antigens, CD34 ,Tretinoin ,Middle Aged ,Prognosis ,Polymerase Chain Reaction ,Disease-Free Survival ,Neoplasm Proteins ,Leukocyte Count ,Treatment Outcome ,Leukemia, Promyelocytic, Acute ,Child, Preschool ,Antineoplastic Combined Chemotherapy Protocols ,Humans ,Protein Isoforms ,Female ,Child ,Aged ,Proportional Hazards Models - 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 counts10 x 10(9)/l (P0.05). The S-form cases displayed a significantly higher number of cases with M3v microgranular features (P = 0.005) and CD34 expression (P0.0001). 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.
- Published
- 2001