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伴有7号染色体单体的费城染色体阳性成人急性白血病:对治疗反应不佳的一个亚组。

Philadelphia chromosome-positive adult acute leukemia with monosomy of chromosome number seven: a subgroup with poor response to therapy.

作者信息

Maddox A M, Keating M J, Trujillo J, Cork A, Youness E, Ahearn M J, McCredie K B, Freireich E J

出版信息

Leuk Res. 1983;7(4):509-22. doi: 10.1016/0145-2126(83)90046-2.

Abstract

Thirty-four adult patients were seen at the University of Texas M. D. Anderson Hospital and Tumor Institute at Houston, Texas between 1969 and 1980 with acute leukemia (AL) and a deleted G-group chromosome that was shown by Giemsa banding to be a Philadelphia (Ph1) chromosome t(9;22) in 21 patients. Fourteen had the Ph1 chromosome as the sole abnormality, 12 had the Ph1 chromosome and loss of one chromosome of the C-group (identified by Giemsa banding analysis as number 7 in eight patients), while eight had the Ph1 chromosome and other changes. These three groups were similar in sex, age distribution and hematologic parameters. The median age of 40 was lower than usually seen in AL. The distribution of the morphologic subtypes was similar to that seen at this institution, with 50% being acute myeloblastic, 12% acute myelomonocytic, 20% lymphoblastic and 18% acute undifferentiated. The complete remission rate with chemotherapy was low: 25% in the Ph1 +/- 7, 50% in the Ph1 +/other group and 43% in the Ph1 +/other group. Median survival time was 8 months for the Ph1 +/- 7 group, 5.5 months for the Ph1 +/other group and 9.0 months for the Ph1 +/alone group. These patients with Ph1 + AL had higher white blood cell counts, increased extramedullary disease and poorer responses to therapy than usual for patients with AL. The deletion of chromosome 7 and the acquisition of the Ph1 chromosome identifies a group of patients with characteristics similar to all the patients with Ph1 + AL but a poor response to therapy and short remission duration.

摘要

1969年至1980年间,在得克萨斯州休斯敦的得克萨斯大学MD安德森医院和肿瘤研究所,对34例成年急性白血病(AL)患者进行了观察,其中21例患者经吉姆萨显带显示,其G组染色体缺失为费城(Ph1)染色体t(9;22)。14例患者的Ph1染色体为唯一异常,12例患者有Ph1染色体且C组中的一条染色体缺失(经吉姆萨显带分析,8例患者为7号染色体),8例患者有Ph1染色体及其他改变。这三组患者在性别、年龄分布和血液学参数方面相似。40岁的中位年龄低于AL患者的常见年龄。形态学亚型的分布与该机构所见相似,急性髓细胞性白血病占50%,急性粒单核细胞性白血病占12%,淋巴细胞性白血病占20%,急性未分化白血病占18%。化疗后的完全缓解率较低:Ph1 +/- 7组为25%,Ph1 +/其他组为50%,Ph1 +/单独组为43%。Ph1 +/- 7组的中位生存时间为8个月,Ph1 +/其他组为5.5个月,Ph1 +/单独组为9.0个月。这些Ph1 + AL患者的白细胞计数较高,髓外疾病增加,对治疗的反应比AL患者通常情况更差。7号染色体缺失和Ph1染色体的获得确定了一组患者,其特征与所有Ph1 + AL患者相似,但对治疗反应不佳且缓解期短。

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