Myelodysplastic syndromes associated with 11q23 abnormalities

被引:32
作者
Bain, BJ
Moorman, AV
Johansson, B
Mehta, AB
Secker-Walker, LM
机构
[1] St Marys Hosp, Imperial Coll, Sch Med, Dept Haematol, London W2 1NY, England
[2] Royal Free Hosp, Sch Med, Dept Haematol, London NW3 2QG, England
[3] Univ Lund Hosp, Dept Clin Genet, S-22185 Lund, Sweden
关键词
myelodysplastic syndromes; cytogenetics; 11q23 chromosomal rearrangements;
D O I
10.1038/sj.leu.2401020
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
Most hematological neoplasms associated with 11q23 chromosomal rearrangement have been cases of acute lymphoblastic or acute myeloid leukemia. Although cases of myelodysplastic syndrome (MDS) are a minority, these syndromes should also be recognized as part of the spectrum of hematological disorders associated with 11q23 rearrangement. In this series of 550 patients with such rearrangement there were 28 (5.1%) who presented with MDS. A further five patients had a history of MDS but had evolved to AML by the time cytogenetic analysis was first undertaken. There were thus a total of 33 patients (6.0%) with an 11q23 abnormality whose initial presentation was as MDS. Of these 33 patients, a quarter (seven patients) were cases of secondary (therapy-related) MDS. Complex karyotypes and other poor prognosis chromosomal abnormalities such as -7 and 7q- were common and were not confined to secondary cases. The likelihood of presentation as MDS differed between different cytogenetic categories. The 28 patients in whom cytogenetic analysis was performed at presentation as MDS showed a wide age distribution, from 1 to 82 years; there were four children, two of 1 year of age. All FAB types of MDS were represented. Median survival was only 19.1 months. Leukemic transformation occurred in five patients including one case of transformation to a biphenotypic M5a/T-lineage acute leukemia.
引用
收藏
页码:834 / 839
页数:6
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