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Original Article
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Korean J Med. 2008;75(4):412-419.
- 원저: 급성전골수구성백혈병에서 PML/RARα 유전자 이형의 임상적 의의
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이원식.이상민.이규형.이제환.최성준.이정희.김대영.임성남.박재후.민영주.김 혁.배성화.류헌, , , , , , , , , , , ,
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- Clinical significance of PML/RARα isoforms in acute promyelocytic leukemia
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Won Sik Lee, Sang Min Lee, Kyoo-Hyung Lee, Je-Hwan Lee, Seong-Joon Choi, Jung-Hee Lee, Dae-Young Kim, Sung-Nam Lim, Jae Hoo Park, Young Joo Min, Hawk Kim, Sung Hwa Bae, Hun
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한양대학교 의과대학 호흡기내과
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- Abstract
- 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.
(Korean J Med 75:412-419, 2008)
Keywords :Acute promyelocytic leukemia, PML-RARα, Isoform
