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具有四倍体克隆且以两个t(15;17)为特征的低细胞性急性早幼粒细胞白血病。

Hypocellular acute promyelocytic leukemia with a tetraploid clone characterized by two t(15;17).

作者信息

Kojima Kensuke, Imaoka Michiyoshi, Noguchi Toshiyuki, Narumi Hiroshi, Uchida Naoyuki, Sakai Ikuya, Yasukawa Masaki, Fujita Shigeru

机构信息

The First Department of Internal Medicine, Ehime University School of Medicine, Shingenobu, Ehime 791-0295, Japan.

出版信息

Cancer Genet Cytogenet. 2003 Sep;145(2):169-71. doi: 10.1016/s0165-4608(03)00097-9.

Abstract

We describe a case of hypocellular acute promyelocytic leukemia with a tetraploid clone characterized by two t(15;17). The large leukemia cells had a bizarre nuclear configuration and multiple Auer rods. A bone marrow biopsy specimen revealed a markedly hypocellular marrow (<10% cellularity) in the absence of myelofibrosis. Myelodysplastic features were not detected. Chromosome analysis of marrow cells revealed a karyotype of 92,XXYY,del(2)(q?),t(15;17)(q22;q21)x2. Interphase fluorescence in situ hybridization revealed that the marrow cells were composed of a tetraploid clone carrying double t(15;17) and normal diploid cells. The leukemia responded well to all-trans retinoic acid. We think that the tetraploidy could be caused by endoreduplication or endomitosis of the diploid clone with single t(15;17). The unique karyotype largely contributed to the cell morphology and marrow hypoplasia, while it may not have affected on the prognosis of the acute promyelocytic leukemia.

摘要

我们描述了一例伴有四倍体克隆的低细胞性急性早幼粒细胞白血病,其特征为两个t(15;17)。大的白血病细胞具有奇异的核形态和多个Auer小体。骨髓活检标本显示骨髓明显低细胞(细胞含量<10%)且无骨髓纤维化。未检测到骨髓增生异常特征。骨髓细胞的染色体分析显示核型为92,XXYY,del(2)(q?),t(15;17)(q22;q21)×2。间期荧光原位杂交显示骨髓细胞由携带双t(15;17)的四倍体克隆和正常二倍体细胞组成。该白血病对全反式维甲酸反应良好。我们认为四倍体可能是由带有单个t(15;17)的二倍体克隆的核内复制或核内有丝分裂引起的。独特的核型在很大程度上导致了细胞形态和骨髓发育不全,而它可能并未影响急性早幼粒细胞白血病的预后。

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