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Biphenotypic acute leukemia with t(15;17) lacking promyelocytic-retinoid acid receptor α rearrangement

机译:t(15; 17)缺乏早幼粒细胞-类维生素A受体重排的双表型急性白血病

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Biphenotypic acute leukemias (BAL) account for less than 4% of all cases of acute leukemia. Philadelphia chromosome and 11q23 rearrangement are the most frequently found cytogenetic abnormalities. Since t(15;17) is almost always associated with acute promyelocytic leukemia, t(15;17) in BAL cases is extremely uncommon. We report here a rare and instructive case of BAL with t(15;17) and the successful treatment approach adopted. A 55-year old woman was referred to our hospital for an examination of elevated white blood cell (WBC) counts with blasts (WBC 13.4×109/L; 76% blasts). The blasts with acute lymphoblastic leukemia (ALL-L2, FAB) morphology coexpressed B-lymphoid and myeloid lineages, and a cytogenetic study revealed 4q21 abnormalities and t(15;17). However, promyelocytic-retinoid acid receptor α rearrangement was not detected by fluorescence in situ hybridization on interphase nuclei. Our patient was treated with chemotherapy for ALL and gemtuzumab ozogamicin without all- trans -retinoic acid, and has remained in hematologic first complete remission for more than 3.7 years.
机译:双表型急性白血病(BAL)占所有急性白血病病例的不到4%。费城染色体和11q23重排是最常见的细胞遗传异常。由于t(15; 17)几乎总是与急性早幼粒细胞白血病相关,因此在BAL病例中t(15; 17)非常罕见。我们在这里报告了一种罕见且具有指导意义的BAL案例,其t(15; 17)并采用了成功的治疗方法。一名55岁的妇女被转诊到我院检查,发现白细胞计数升高(WBC 13.4×109 / L; 76%原始细胞)。急性淋巴细胞白血病(ALL-L2,FAB)形态的胚细胞共表达B淋巴和髓系,并且细胞遗传学研究显示4q21异常和t(15; 17)。然而,通过相间核上的荧光原位杂交未检测到早幼粒细胞-类维生素A受体α重排。我们的患者接受了ALL和吉妥单抗ozogamicin的化学疗法,不含全反式维甲酸,并且在血液学上首次完全缓解已超过3.7年。

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