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伴有t(9;11)(p21-22;q23)的血液系统恶性肿瘤——125例的实验室及临床研究。欧洲11q23研讨会参与者

Hematological malignancies with t(9;11)(p21-22;q23)--a laboratory and clinical study of 125 cases. European 11q23 Workshop participants.

作者信息

Swansbury G J, Slater R, Bain B J, Moorman A V, Secker-Walker L M

机构信息

Academic Haematology and Cytogenetics, The Royal Marsden NHS Trust, Sutton, UK.

出版信息

Leukemia. 1998 May;12(5):792-800. doi: 10.1038/sj.leu.2401014.

DOI:10.1038/sj.leu.2401014
PMID:9593283
Abstract

This paper reports clinical and cytogenetic data from 125 cases with t(9;11)(p21-22;q32) which were accepted for a European Union Concerted Action Workshop on 11q23. This chromosome abnormality is known to occur predominantly in acute myeloid leukemia (AML) FAB type M5a and less often in AML M4; in this series it was also found to occur, uncommonly, in other AML FAB types, in childhood acute lymphoblastic leukemia (ALL) (nine cases), in relatively young patients with myelodysplastic syndrome (MDS) (five cases), acute biphenotypic leukemia (two cases), and acute undifferentiated leukemia (one case). All age groups were represented but 50% of the patients were aged less than 15 years. The t(9;11) was the sole abnormality in 57 cases with AML; trisomy 8 was the most common additional abnormality (23 cases, including seven with further abnormalities), and 28 cases had other additional abnormalities. Among the t(9;11)+ve patients with AML, the white cell count (WBC) and age group were significant predictors of event-free survival; central nervous system (CNS) involvement or karyotype class (sole, with trisomy 8, or with other), also contributed to prognosis although our data could not show these to be independent factors. The best outcome was for patients aged 1-9 years, with low WBC, and with absence of CNS disease or presence of trisomy 8. For patients aged less than 15 years, the event-free survival for ALL patients was not significantly worse than that of AML patients.

摘要

本文报告了125例t(9;11)(p21 - 22;q32)患者的临床和细胞遗传学数据,这些病例被纳入了欧盟关于11q23的联合行动研讨会。已知这种染色体异常主要发生在急性髓系白血病(AML)FAB分型的M5a型中,较少见于AML M4型;在本系列研究中,还发现它罕见地发生于其他AML FAB分型、儿童急性淋巴细胞白血病(ALL)(9例)、相对年轻的骨髓增生异常综合征(MDS)患者(5例)、急性双表型白血病(2例)以及急性未分化白血病(1例)。各年龄组均有代表,但50%的患者年龄小于15岁。t(9;11)是57例AML患者的唯一异常;8号染色体三体是最常见的附加异常(23例,包括7例有进一步异常的),28例有其他附加异常。在t(9;11)阳性的AML患者中,白细胞计数(WBC)和年龄组是无事件生存的重要预测因素;中枢神经系统(CNS)受累或核型类别(单独、伴有8号染色体三体或其他)也对预后有影响,尽管我们的数据未能表明这些是独立因素。最佳预后见于年龄在1 - 9岁、WBC低且无CNS疾病或有8号染色体三体的患者。对于年龄小于15岁的患者,ALL患者的无事件生存并不比AML患者显著更差。

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