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Keywords:

  • acute promyelocytic leukaemia;
  • PML/RARα isoform;
  • clinical outcome;
  • complete remission;
  • all-trans retinoic acid (ATRA);
  • reverse transcription–polymerase chain reaction (RT–PCR)

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 × 109/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.