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KMID : 0387820140210020071
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Clinical Pediatric Hematology-Oncology
2014 Volume.21 No. 2 p.71 ~ p.79
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Prevalence and Clinical Implication of Partial Tandem Duplication of the Mixed Lineage Leukemia Gene in Pediatric Acute Leukemia
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Jin Song-Lee
Hahn Seung-Min
Kim Hyo-Sun
Lyu Chuhl-Joo
Han Jung-Woo
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Abstract
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Background: The mixed lineage leukemia (MLL) gene may induce hematopoiesis and leukemia. Partial tandem duplication of MLL (MLL-PTD) is associated with poor prog-nosis in acute myeloid leukemia (AML); however, the significance of MLL-PTD in acute lymphoblastic leukemia (ALL) has not been thoroughly studied. We evaluated the in-cidence, relationship with other cytogenetic abnormalities, and the prognostic role of MLL-PTD in ALL.
Methods: We reviewed medical records from pediatric patients diagnosed with ALL in Severance Hospital, Yonsei University Health System, South Korea from 2002 to 2008. MLL-PTD was detected by nested reverse transcriptase polymerase chain reaction.
Results: In ALL patients, 50.0% (42/84) were positive for MLL-PTD. There was no sig-nificant difference in the 10-year overall survival (10Y OS) and event-free survival (EFS) between MLL-PTD-positive (+) and MLL-PTD-negative (?) groups (69.4% vs. 76.2%, P=0.609, and 62.6% vs. 66.7%, P=0.818, respectively). The combination of high level of lactate dehydrogenase (＞1,100 IU/L) and MLL-PTD(+) [MLL-PTD(+)/High LDH] was a statistically significant negative prognostic factor for 10Y OS and EFS (P=0.0226 and P=0.0230, respectively). In multivariate analysis, National Cancer Institute risk strat-ification and very high risk features were independent significant prognostic factors but MLL-PTD (+)/High LDH was not.
Conclusion: MLL-PTD was observed frequently in pediatric ALL patients. MLL-PTD was not an independent prognostic factor. MLL-PTD (+)/High LDH should be evaluated fur-ther for its prognostic potential in ALL.
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KEYWORD
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Acute leukemia, Cytogenetics, Partial tandem duplication, MLL, Prognostic factors, Pediatric leukemia
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