TY - JOUR
T1 - Trisomy 11 in myelodysplastic syndromes defines a unique group of disease with aggressive clinicopathologic features
AU - Wang, S. A.
AU - Jabbar, K.
AU - Lu, G.
AU - Chen, S. S.
AU - Galili, N.
AU - Vega, F.
AU - Jones, D.
AU - Raza, A.
AU - Kantarjian, H.
AU - Garcia-Manero, G.
AU - McDonnell, T. J.
AU - Medeiros, L. J.
PY - 2010
Y1 - 2010
N2 - 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 (n10) or associated with one or two additional alterations (n7), 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 (P0.0002), but comparable to the poor-risk group (P0.97). 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.
AB - 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 (n10) or associated with one or two additional alterations (n7), 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 (P0.0002), but comparable to the poor-risk group (P0.97). 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.
KW - Acute myeloid leukemia
KW - MLL partial tandem duplication
KW - Myelodysplastic syndrome
KW - Overall survival
KW - Trisomy 11
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U2 - 10.1038/leu.2009.289
DO - 10.1038/leu.2009.289
M3 - Article
C2 - 20072149
AN - SCOPUS:77950920130
SN - 0887-6924
VL - 24
SP - 740
EP - 747
JO - Leukemia
JF - Leukemia
IS - 4
ER -