Clinical, morphological, cytogenetic, and prognostic features of patients with myelodysplastic syndromes and del(5q) including band q31

被引:155
作者
Giagounidis, AAN
Germing, U
Haase, S
Hildebrandt, B
Schlegelberger, B
Schoch, C
Wilkens, L
Heinsch, M
Willems, H
Aivado, M
Aul, C
机构
[1] St Johannes Hosp, Med Klin 2, D-47166 Duisburg, Germany
[2] Univ Dusseldorf, Klin Hamatol Onkol & Klin Immunol, D-4000 Dusseldorf, Germany
[3] Univ Dusseldorf, Inst Humangenet, D-4000 Dusseldorf, Germany
[4] Hannover Med Sch, Inst Zell & Mol Pathol, Hannover, Germany
[5] Univ Munich, Lab Spezielle Leukamie Diagnost, Munich, Germany
关键词
5q-syndrome; morphology; cytogenetics; prognosis;
D O I
10.1038/sj.leu.2403189
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
We analyzed data of 76 consecutive patients with myelodysplastic syndrome (MDS) and isolated del(5q) (n=66) or del(5q) plus one additional chromosomal abnormality (n=10) included in our MDS database over the last 26 years. The median age of our patient population was 66.8 years. The male to female ratio was 1:1.7. In all, 14 patients (18%) had advanced MDS with an increased medullary blast count. A total of 17 patients (22%) had significant dysplasia in the nonmegakaryocytic cell lines. Nearly half of the study population showed erythroid hypoplasia in the bone marrow. The projected median survival of patients with isolated del(5q) is 146 months for a median follow-up of 67 months. Patients with an increased medullary blast count and those with an additional chromosomal abnormality have a significantly shorter overall survival (24 and 45 months, respectively) than patients with isolated del(5q). We did not find survival differences for different cytogenetic breakpoints, nor did the amount of dysplasia have an impact on survival in our population. In total, 29 patients have died. Deaths occurred primarily due to transformation into acute leukemia, infection, or cardiac failure. Our data support the current definition of a separate entity of MDS with del(5q) that has been suggested by the World Health Organization.
引用
收藏
页码:113 / 119
页数:7
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