Trisomy 11 in Myelodysplastic Syndromes Defines a Unique Group with Aggressive Clinicopathologic Features
SA Wang, K Jabbar, G Lu, TJ McDonnell, LJ Medeiros. UT MD Anderson Cancer Center, Houston, TX
Background: Trisomy 11 (+11) in acute myeloid leukemia (AML) commonly harbors MLL gene partial tandem duplication (MLL-PTD) and correlates with clinical aggressiveness. The significance of +11 in myelodysplastic syndromes (MDS), however, is largely unknown. It is currently assigned to the intermediate risk group by the international prognostic scoring system (IPSS).
Design: We searched the data files of three large hospitals over a 15-year period for cases of MDS with +11 as a sole cytogenetic abnormality or part of a non-complex karyotype, and compared these cases to patients with AML with +11 from the same hospitals.
Results: We identified 17 MDS patients, in which +11 was present as a sole abnormality(n=10) or associated with 1 or 2 additional abnormalities (n=7), showing a 0.3% frequency in all MDS. 14 of 16 (88%) patients showed excess blasts at presentation, 69% patients evolved to AML at a 5 month median interval, and the median overall survival (OS) was 14 months. For comparison, we studied 19 AML patients with +11 in a non-complex karyotype, of which a substantial subset of patients showed morphological evidence of dysplasia, and/or preexisting cytopenia(s)/MDS. PCR analysis of genomic DNA demonstrated MLL-PTD in 5/10 MDS and 7/11 AML patients.
Our literature review identified 17 cases of MDS with +11, and these cases demonstrated strikingly similar clinicopathologic features to our patients. Compared to our historical data comprising 1165 MDS patients, patients with +11 showed an OS significantly shorter than other patients in the IPSS intermediate risk cytogenetic group(p=0.0002), but comparable to patients in the poor risk group (p=0.97).
Conclusions: Trisomy 11 in MDS correlates with an aggressive clinical course, may suggest an early/evolving AML with MDS-related changes, and is best considered a high-risk cytogenetic abnormality in the prognostication of MDS patients.
Wednesday, March 24, 2010 1:00 PM
Poster Session VI # 190, Wednesday Afternoon