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伴有8号与21号染色体易位的急性髓系白血病。法国血液细胞遗传学小组关于148例病例的报告。

Acute myelogenous leukemia with an 8;21 translocation. A report on 148 cases from the Groupe Français de Cytogénétique Hématologique.

出版信息

Cancer Genet Cytogenet. 1990 Feb;44(2):169-79.

PMID:2297675
Abstract

A retrospective study of 148 previously untreated patients with acute nonlymphocytic leukemia (ANLL) presenting a t(8;21) was undertaken by the Groupe Français de Cytogénétique Hématologique (GFCH). The mean age was 30.8 years for 33 children and 115 adults, 80% of patients were under 50 years, and 66% were males. The sex ratio was unbalanced only in adults (p less than 0.05). Morphologic diagnosis was M2 in 92% of patients. Normal and abnormal mitoses were found in 45% of cases. Complex variant translocations involving 8q22, 21q22, and another chromosome had a frequency of 3.4%. In 75% of the cases additional chromosomal abnormalities were observed. Sex chromosome loss was found (73% of additional abnormalities) in 41% of females and 61% of males. Trisomy 8 was the other recurrent numerical abnormality (7.5%). Chromosome 9 was frequently involved in additional abnormalities (11%), mainly in deletions overlapping the region 9q21-22. Deletions or translocations of chromosome 7(q) were observed in 10% of the cases. The order of appearance did not follow a precise pattern. The remission rate was 90.7%. It was similar in males and females, children and adults. The median survival duration from diagnosis was 17.5 months, with a 24% probability of 5 year survival. Children had a median survival of 24 months from diagnosis, which is to be compared to 16 months for the adults (not statistically different). In no cytogenetic category was a white cell count level higher than 10 x10(9)/L associated with a poorer prognosis. It was concluded that despite the high complete remission rate in t(8;21) ANLL, when a comparison is made between patients achieving a complete remission, the 17-month median survival is similar to that reported in recently published series of ANLL.

摘要

法国血液细胞遗传学小组(GFCH)对148例既往未经治疗的急性非淋巴细胞白血病(ANLL)伴t(8;21)患者进行了一项回顾性研究。33名儿童和115名成人的平均年龄为30.8岁,80%的患者年龄在50岁以下,66%为男性。性别比仅在成人中失衡(p小于0.05)。92%的患者形态学诊断为M2。45%的病例中发现正常和异常有丝分裂。涉及8q22、21q22和另一条染色体的复杂变异易位发生率为3.4%。75%的病例观察到额外的染色体异常。41%的女性和61%的男性存在性染色体丢失(占额外异常的73%)。三体8是另一种常见的数目异常(7.5%)。染色体9经常参与额外异常(11%),主要是9q21 - 22区域重叠的缺失。10%的病例观察到染色体7(q)的缺失或易位。出现顺序没有遵循精确模式。缓解率为90.7%。男性和女性、儿童和成人相似。诊断后的中位生存时间为17.5个月,5年生存率为24%。儿童诊断后的中位生存时间为24个月,而成人为16个月(无统计学差异)。在任何细胞遗传学类别中,白细胞计数水平高于10×10(9)/L与预后较差无关。结论是,尽管t(8;21) ANLL的完全缓解率较高,但在达到完全缓解的患者之间进行比较时,17个月的中位生存时间与最近发表的ANLL系列报道相似。

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