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KMID : 0882420080750040412
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Korean Journal of Medicine
2008 Volume.75 No. 4 p.412 ~ p.419
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Clinical significance of PML/RARα isoforms in acute promyelocytic leukemia
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Lee Won-Sik
Lee Sang-Min
Lee Kyoo-Hyung
Lee Je-Hwan
Choi Seong-Jun
Lee Jung-Hee
Kim Dae-Young
Lim Sung-Nam
Park Jae-Hoo
Kim Hawk
Bae Sung-Hwa
Ryoo Hun-Mo
Hyun Myung-Soo
Zang Dae-Young
Kim Hyo-Jung
Jung Chul-Won
Ahn Jin-Seok
Lee Gyeong-Won
Lee Jung-Lim
Joo Young-Don
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Abstract
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Background/Aims: There are three types of PML-RARα mRNA fusion transcripts associated with acute promyelocytic leukemia (APL): the short (S)-form, the long (L)-form and the variable (V)-form. No study on the Korean population has addressed the clinical significance of the specific types of PML-RARα mRNA fusion transcripts for APL patients who receive the combination therapy of all-trans-retinoic-acid and idarubicin (AIDA regimen).
Methods: We performed a retrospective analysis on 94 patients with APL to evaluate differences in the therapeutic
outcomes, such as the response rate, an event-free survival (EFS), and overall survival (OS), after remission following the
induction of chemotherapy. We also analyzed whether differences in the pretreatment clinical characteristics depend on the PML-RARα isoform.
Results: The median age of the patients was 41 years (range 15-85). Among the 94 patients, there were 58 L-form
cases (62.1%), 32 S-form cases (34.0%), and 4 V-form cases (4.3%). The CR rate following remission induction treatment
was 84.9%. The CR rate was higher in patients with an initial WBC <10.0×109/L, as compared to patients with an initial
WBC higher than 10.0×109/L (93.5% vs. 65.4%, p=0.001). The AIDA induction regimen was associated with a better
EFS than non-AIDA induction regimens (81.9% vs. 49.6%, p=0.006). The induction group was also a significant prognostic factor for EFS in the multivariate analysis (p=0.020). There were no differences in OS and EFS in patients with either isoform L or isoform S in the AIDA induction group.
Conclusions: This retrospective study demonstrated that pretreatment clinical characteristics and treatment outcomes were not significantly different among patients with varying PML-RARα isoform types in the AIDA induction group.
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KEYWORD
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Acute promyelocytic leukemia, PML-RARα, Isoform
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