关键词: acute myeloid leukemia acute promyelocytic leukemia basophils cytogenetic translocation treatment

来  源:   DOI:10.3390/hematolrep16020037   PDF(Pubmed)

Abstract:
Acute promyelocytic leukemia is a rare form of acute myeloid leukemia in which immature promyelocytes abnormally proliferate in the bone marrow. In most cases, the disease is characterised by the translocation t(15;17) (q24;q21), which causes the formation of PML::RARA, an oncogenic fusion protein responsible for blocking myeloid differentiation and survival advantage. Here, we present a case of acute promyelocytic leukemia with two unusual features: basophilic differentiation and a three-way translocation involving chromosomes 12, 15 and 17. In the few cases reported, basophilic differentiation was associated with a poor prognosis. In contrast, our patient responded promptly to the standard treatment with all-trans-retinoic acid (ATRA) and arsenic trioxide (ATO) and obtained complete remission. To our knowledge, this is the first report of basophilic acute promyelocytic leukemia with the three-way translocation t(12;17;15) (p13; q24;q21).
摘要:
急性早幼粒细胞白血病是一种罕见的急性髓性白血病,其中未成熟的早幼粒细胞在骨髓中异常增殖。在大多数情况下,该疾病的特征是易位t(15;17)(q24;q21),这导致了PML::RARA的形成,负责阻断骨髓分化和生存优势的致癌融合蛋白。这里,我们介绍了一例急性早幼粒细胞白血病,具有两个不寻常的特征:嗜碱性细胞分化和涉及染色体12、15和17的三向易位。在报告的少数案例中,嗜碱性细胞分化与不良预后相关。相比之下,我们的患者对全反式维甲酸(ATRA)和三氧化二砷(ATO)的标准治疗反应迅速,并获得完全缓解.据我们所知,这是三向易位t(12;17;15)(p13;q24;q21)的嗜碱性急性早幼粒细胞白血病的首次报道。
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