Clinical significance of PML/RAR alpha isoforms in acute promyelocytic leukemia.
- Author:
Won Sik LEE
1
;
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 Mo RYOO
;
Myung Soo HYUN
;
Min Kyung KIM
;
Dae Young ZANG
;
Hyo Jung KIM
;
Chul Won JUNG
;
Jin Seok AHN
;
Kyeong Won LEE
;
Jung Lim LEE
;
Young Don JOO
Author Information
1. Department of Internal Medicine, Inje University College of Medicine1, Busan Paik Hospital, Pusan, Korea. yjoo@inje.ac.kr
- Publication Type:Original Article
- Keywords:
Acute promyelocytic leukemia;
PML-RAR alpha;
Isoform
- MeSH:
Disease-Free Survival;
Humans;
Idarubicin;
Leukemia, Promyelocytic, Acute;
Multivariate Analysis;
Protein Isoforms;
Remission Induction;
Retrospective Studies;
RNA, Messenger
- From:Korean Journal of Medicine
2008;75(4):412-419
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
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.
