Trisomy 11 in myelodysplastic syndromes defines a unique group of disease with aggressive clinicopathologic features

ABSTRACT Trisomy 11 in myelodysplastic syndromes (MDS) is rare, with undefined clinical significance and is currently assigned to the International Prognostic Scoring System (IPSS)

intermediate-risk group. Over a 15-year period, we identified 17 MDS patients with trisomy 11 either as a sole abnormality (_n_=10) or associated with one or two additional alterations

(_n_=7), comprising 0.3% of all MDS cases reviewed. Of 16 patients with Bone Marrow material available for review, 14 (88%) patients presented with excess blasts, 69% patients evolved to

acute myeloid leukemia (AML) in a 5-month median interval and the median survival was 14 months. For comparison, we studied 19 AML patients with trisomy 11 in a noncomplex karyotype, of

which, a substantial subset of patients had morphologic dysplasia, and/or preexisting cytopenia(s)/MDS. Genomic DNA PCR showed _MLL_ partial tandem duplication in 5 of 10 MDS and 7 of 11 AML

patients. A review of literature identified 17 additional cases of MDS with trisomy 11, showing similar clinicopathologic features to our patients. Compared with our historical data

comprising 1165 MDS patients, MDS patients with trisomy 11 had a significantly inferior survival to patients in the IPSS intermediate-risk cytogenetic group (_P_=0.0002), but comparable to

the poor-risk group (_P_=0.97). We conclude that trisomy 11 in MDS correlates with clinical aggressiveness, may suggest an early/evolving AML with myelodysplasia-related changes and is best

considered a high-risk cytogenetic abnormality in MDS prognostication. Access through your institution Buy or subscribe This is a preview of subscription content, access via your institution

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AUTHORS AND AFFILIATIONS * Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA S A Wang, K Jabbar, G Lu, S S Chen, F Vega, D Jones, T J

McDonnell & L J Medeiros * Department of Internal Medicine, St Vincent's Comprehensive Cancer Center, New York, NY, USA N Galili & A Raza * Department of Leukemia, The

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Wang. ETHICS DECLARATIONS COMPETING INTERESTS The authors declare no conflict of interest. ADDITIONAL INFORMATION Supplementary Information accompanies the paper on the Leukemia website

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Jabbar, K., Lu, G. _et al._ Trisomy 11 in myelodysplastic syndromes defines a unique group of disease with aggressive clinicopathologic features. _Leukemia_ 24, 740–747 (2010).

https://doi.org/10.1038/leu.2009.289 Download citation * Received: 09 November 2009 * Accepted: 23 November 2009 * Published: 14 January 2010 * Issue Date: April 2010 * DOI:

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currently available for this article. Copy to clipboard Provided by the Springer Nature SharedIt content-sharing initiative KEYWORDS * myelodysplastic syndrome * trisomy 11 * acute myeloid

leukemia * overall survival * _MLL_ partial tandem duplication