Pretreatment characteristics and clinical outcome of acute promyelocytic leukaemia patients according to the PML-RAR alpha isoforms: a study of the PETHEMA group

Br J Haematol. 2001 Jul;114(1):99-103. doi: 10.1046/j.1365-2141.2001.02915.x.

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.

Publication types

  • Clinical Trial
  • Comparative Study
  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Antigens, CD34 / analysis
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Child
  • Child, Preschool
  • Disease-Free Survival
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Leukemia, Promyelocytic, Acute / drug therapy
  • Leukemia, Promyelocytic, Acute / immunology
  • Leukemia, Promyelocytic, Acute / metabolism*
  • Leukocyte Count
  • Male
  • Middle Aged
  • Neoplasm Proteins / genetics*
  • Oncogene Proteins, Fusion / genetics*
  • Polymerase Chain Reaction / methods
  • Prognosis
  • Proportional Hazards Models
  • Protein Isoforms / genetics
  • Treatment Outcome
  • Tretinoin / therapeutic use

Substances

  • Antigens, CD34
  • Neoplasm Proteins
  • Oncogene Proteins, Fusion
  • Protein Isoforms
  • promyelocytic leukemia-retinoic acid receptor alpha fusion oncoprotein
  • Tretinoin