Publication Date:
2010-11-19
Description:
Abstract 2930 MDS is characterized by recurrent chromosomal abnormalities that reflect the underlying genomic instability of the disease and help to predict clinical prognosis. Abnormalities of chromosome 17 have been described previously in MDS and AML and may have some association to prior exposure to cytotoxic therapy. Abnormalities of the p53 gene, which is located in the short arm of chromosome 17 (17p), have been discovered in the non-deleted allele in a large proportion these cases – suggesting an association and likely contributing to the pathogenesis of this subgroup. Here we present the clinical characteristics, prognosis, and response to therapy of patients (pts) with MDS with and without chromosome 17 abnormalities in our single center experience. We analyzed our records for cases of MDS referred to MD Anderson Cancer Center from 2000–2009. Of the 1638 cases of MDS examined, 88 (5.4%) had evidence of any chromosome 17 abnormality (abnl). The specific chromosome 17 abnormalities were: 17p- in 8 (9%); der 17 in 1 (1%); -17 in 78 (89%); and 17p-, -17 in 1 (1%). In terms of the complete karyotype, the pts could be grouped as: 17 abnl with complex (95%), 17 abnl with -5 or -7 (94%), and 17 abnl non-complex (5%). There were no cases with an isolated 17 abnl. The median age of these pts with chromosome 17 abnormality (cohort A) was 67 yrs (range 16–87), compared to 67 (13-94) in the pts without chromosome 17 abnormality (cohort B, n=1550). The MDS diagnoses by WHO in cohort A were RA, MDS-U in 23 (26%), RAEB in 64 (73%), and 5q- in 1 (1%) compared with 47%, 52%, and 1% in cohort B respectively (p=0.001). Stratification by IPSS (International Prognostic Scoring System) in cohort A revealed 0 (0%) with low risk, 8 (9%) with intermediate-1 (INT-1), 56 (64%) with INT-2, and 20 (23%) with high risk compared with 19%, 40%, 29%, and 9% in the respective IPSS groups in cohort B (p
Print ISSN:
0006-4971
Electronic ISSN:
1528-0020
Topics:
Biology
,
Medicine
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