混合表型急性白血病伴骨髓坏死的非典型 BCR-ABL1 转录本。

IF 3 2区 医学 Q3 BIOCHEMISTRY & MOLECULAR BIOLOGY Molecular Carcinogenesis Pub Date : 2024-08-01 Epub Date: 2024-06-11 DOI:10.1002/mc.23742
Jiarui Liu, Yujie Jiang, Dai Yuan, Zhifen Zhang, Xin Liu, Wenbo Zhao, Hongzhi Xu
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引用次数: 0

摘要

混合表型急性白血病(MPAL)是一种急性白血病,其中包含髓系、T淋巴细胞和/或B淋巴细胞分化的混合特征。费城染色体阳性(Ph+)MPAL 是一种罕见的亚群,预后较差,占成人急性白血病的 1%。迄今为止,关于如何最佳治疗 Ph+ MPAL 仍未达成共识。在此,我们报告了一名患有 Ph+(非典型 e13a2 BCR-ABL1 融合蛋白)MPAL 的 62 岁男性患者。该患者因骨髓坏死(BMN)而反复出现剧烈骨痛。此外,他在接受氟马替尼联合超CVAD(B)(一种包括甲氨蝶呤和阿糖胞苷的剂量强化方案)治疗之前,前两次化疗均未取得完全缓解。据我们所知,这是第一份描述MPAL患者同时存在BMN和非典型e13a2 BCR-ABL1转录本的报告。这一发现将为Ph+ MPAL的诊断和治疗带来新的认识。
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Atypical BCR-ABL1 transcript in mixed phenotype acute leukemia with bone marrow necrosis.

Mixed phenotype acute leukemia (MPAL) is a type of acute leukemia in which encompasses mixed features of myeloid, T-lymphoid, and/or B-lymphoid differentiation. Philadelphia chromosome-positive (Ph+) MPAL is a rare subgroup with a poor prognosis and accounts for <1% of adult acute leukemia. Until now, there is still no consensus on how to best treat Ph+ MPAL. Here, we report a 62-year-old male with Ph+ (atypical e13a2 BCR-ABL1 fusion protein) MPAL. This patient presented with recurrent and intense bone pain due to bone marrow necrosis (BMN). Besides, he did not achieve a complete remission for the first two chemotherapies, until he received flumatinib combined with hyper-CVAD (B) (a dose-intensive regimen include methotrexate and cytarabine). To our knowledge, this is the first report to describe the coexistence of BMN and atypical e13a2 BCR-ABL1 transcripts in patients with MPAL. This finding will bring new understandings in the diagnosis and treatment of Ph+ MPAL.

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来源期刊
Molecular Carcinogenesis
Molecular Carcinogenesis 医学-生化与分子生物学
CiteScore
7.30
自引率
2.20%
发文量
112
审稿时长
2 months
期刊介绍: Molecular Carcinogenesis publishes articles describing discoveries in basic and clinical science of the mechanisms involved in chemical-, environmental-, physical (e.g., radiation, trauma)-, infection and inflammation-associated cancer development, basic mechanisms of cancer prevention and therapy, the function of oncogenes and tumors suppressors, and the role of biomarkers for cancer risk prediction, molecular diagnosis and prognosis.
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