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59例急性非淋巴细胞白血病或骨髓增生异常综合征患者伴12号染色体短臂异常的细胞遗传学、分子细胞遗传学及临床发现的相关性

Correlation of cytogenetic, molecular cytogenetic, and clinical findings in 59 patients with ANLL or MDS and abnormalities of the short arm of chromosome 12.

作者信息

Streubel B, Sauerland C, Heil G, Freund M, Bartels H, Lengfelder E, Wandt H, Ludwig W D, Nowotny H, Baldus M, Grothaus-Pinke B, Büchner T, Fonatsch C

机构信息

Institut für Medizinische Biologie der Universität Wien, Austria.

出版信息

Br J Haematol. 1998 Mar;100(3):521-33. doi: 10.1046/j.1365-2141.1998.00591.x.

Abstract

Abnormalities of the short arm of chromosome 12 (12p) are found in about 5% of acute nonlymphocytic leukaemias (ANLL) and myelodysplastic syndromes (MDS). They are described to be characteristic of secondary leukaemias, especially after prior mutagenic exposure, and to be associated with a poor prognosis. In our series of 59 patients with 12p abnormalities and ANLL or MDS, exposure to genotoxic agents was proven only in five patients, but in 13/44 patients ANLL evolved from an MDS. Patients with a small deletion del(12)(p11.2p13) having a mild clinical course were distinguished from those with a large del(12)(p11.2), additional chromosomal anomalies, and a poor clinical course. Among the 31 patients with translocations or dicentric chromosomes involving 12p, a group of eight with t/dic(12;13) was the most frequent and was associated with a poor prognosis. The clinical outcome was adverse in the majority of patients with complex karyotype abnormalities, but in some patients a milder clinical course seems likely. A new, hitherto undescribed, abnormality in an MDS case with a duplication dup(12)(p11.2p13) was the amplification of the signal of the yeast artificial chromosome (YAC) clone 964c10 (D12S736). In 38 cases with deletions or unbalanced translocations/dicentrics one YAC signal was lost. Five patients with balanced translocations demonstrated breakpoints within the YAC, containing the ETV6 (TEL) gene. The breakpoints were telomeric to the YAC 964c10 in seven cases and centromeric in one patient.

摘要

12号染色体短臂(12p)异常见于约5%的急性非淋巴细胞白血病(ANLL)和骨髓增生异常综合征(MDS)。这些异常被认为是继发性白血病的特征,尤其是在先前接触诱变剂之后,并且与预后不良相关。在我们的59例伴有12p异常及ANLL或MDS的患者系列中,仅在5例患者中证实有遗传毒性剂暴露,但在13/44例ANLL患者中,白血病是由MDS演变而来。具有轻度临床病程的小缺失del(12)(p11.2p13)患者与具有大的del(12)(p11.2)、额外染色体异常及不良临床病程的患者有所不同。在31例涉及12p的易位或双着丝粒染色体患者中,一组8例t/dic(12;13)最为常见,且与预后不良相关。大多数核型复杂异常的患者临床结局不良,但部分患者临床病程似乎较轻。1例伴有重复dup(12)(p11.2p13)的MDS病例中,一种新的、迄今未描述的异常是酵母人工染色体(YAC)克隆964c10(D12S736)信号的扩增。在38例有缺失或不平衡易位/双着丝粒的病例中,一个YAC信号丢失。5例平衡易位患者的断点位于包含ETV6(TEL)基因的YAC内。7例患者的断点位于YAC 964c10的端粒侧,1例患者的断点位于着丝粒侧。

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