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伴有PML-RARα阳性的混合表型急性白血病:一例报告及文献复习

Mixed phenotype acute leukemia with PML-RARα positive: a case report and literature review.

作者信息

Zheng Xiaolong, Shen Huafei, Zhu Mingyu, Shi Yuanfei, Wang Huanping, Chen Zhimei, Huang Xin, Wang Yungui, Jin Jie, Xie Wanzhuo

机构信息

Department of Hematology, The First Affiliated Hospital, Zhejiang University School of Medicine, #79 Qingchun Road, Hangzhou, 310003, Zhejiang Province, People's Republic of China.

出版信息

Mol Cytogenet. 2021 Feb 11;14(1):10. doi: 10.1186/s13039-021-00530-9.

DOI:10.1186/s13039-021-00530-9
PMID:33573663
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7879616/
Abstract

Mixed phenotype acute leukemia (MPAL) is an uncommon type of leukemia. It is one kind of malignant clonal diseases that expresses more than one genealogical specific antigen simultaneously. Most MPAL patients are associated with clonal chromosomal abnormalities and molecular genetic changes, such as t(9;22) (q34;q11) and KMT2A (MLL) rearrangement. These specific abnormalities usually have important guiding significance in MPAL diagnosis, targeted therapy and prognosis judgment. In this paper, we reported a case of MPAL, T/myeloid (M5) with an unfrequent combination of PML-RARα positivity and t(15;17). The treatment was successful with chemotherapy for both AML and ALL with daunorubicin, cytarabine (DA) and vincristine, prednisone (VP). We reported here this suggestive MPAL case of rare disease condition and effective treatment, in order to provide experience for the early diagnosis and treatment of similar patients.

摘要

混合表型急性白血病(MPAL)是一种罕见的白血病类型。它是一种恶性克隆性疾病,可同时表达一种以上谱系特异性抗原。大多数MPAL患者与克隆性染色体异常和分子遗传学改变有关,如t(9;22)(q34;q11)和KMT2A(MLL)重排。这些特异性异常通常在MPAL诊断、靶向治疗及预后判断中具有重要的指导意义。本文报道1例MPAL,T/髓系(M5),伴有罕见的PML-RARα阳性和t(15;17)组合。采用柔红霉素、阿糖胞苷(DA)以及长春新碱、泼尼松(VP)对急性髓系白血病(AML)和急性淋巴细胞白血病(ALL)进行化疗,治疗成功。我们在此报告这例具有提示意义的罕见病MPAL病例及有效治疗方法,以便为类似患者的早期诊断和治疗提供经验。

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