Korean J Med.
2008 Oct;75(4):412-419.
Clinical significance of PML/RAR alpha isoforms in acute promyelocytic leukemia
- Affiliations
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- 1Department of Internal Medicine, Inje University College of Medicine1, Busan Paik Hospital, Pusan, Korea. yjoo@inje.ac.kr
- 2Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
- 3Department of Internal Medicine, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Korea.
- 4Department of Internal Medicine, Daegu Catholic University College of Medicine, Daegu, Korea.
- 5Department of Internal Medicine, Yeungnam University College of Medicine, Yeungnam, Korea.
- 6Department of Internal Medicine, Hallym University College of Medicine, Seoul, Korea.
- 7Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
- 8Department of Internal Medicine, College of Medicine, Gyeong-Sang National University, Gyeongsang, Korea.
- 9Department of Internal Medicine, Daegu Patima Hospital, Daegu, Korea.
Abstract
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BACKGROUND/AIMS: There are three types of PML-RAR alpha 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-RAR alpha 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-RAR alpha 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 <10.0x109/L, as compared to patients with an initial WBC higher than 10.0X109/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-RAR alpha isoform types in the AIDA induction group.
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