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1例罕见的具有髓系拟态的儿童T细胞急性淋巴细胞白血病。

A rare case of pediatric T-cell acute lymphoblastic leukemia with myeloid mimicry.

作者信息

Ni Wenpeng, Liu Ji, Deng Kunyi, Zeng Zhouyu, Kong Qingzhu, Weng Xiaoling

机构信息

Clinical Laboratory, Boai Hospital of Zhongshan, No. 6 Chenggui Road, Zhongshan, 528400, Guangdong, China.

出版信息

Discov Oncol. 2024 Nov 21;15(1):691. doi: 10.1007/s12672-024-01600-1.

Abstract

Acute lymphoblastic leukemia (ALL) with granular blasts was historically referred to as "granular ALL", but this is not recognized as a distinct entity in the current WHO classification. This rare morphological feature is predominantly associated with a B-cell immunophenotype, while T-ALL with prominent cytoplasmic granules is extremely rare, with limited available genetic, molecular, and prognostic data. Here, we report a unique case of ALL in a 9-year-old girl. Initial blood tests revealed leukocytosis, and both peripheral blood and bone marrow morphology showed a high number of blasts with granular cytoplasm and bilobed nuclei. Immunophenotyping confirmed T-cell origin. The patient tested positive for KMT2A::AFDN, with WT1 overexpression, and NRAS and EZH2 mutations were detected by next-generation sequencing (NGS). Remarkably, the patient achieved complete remission after treatment and has shown no signs of relapse for nearly four years. Her favorable response to conventional therapy underscores the importance of molecular phenotyping in the treatment of this disease. The continued use of NGS to gather relevant molecular data is crucial for further understanding the molecular phenotype and prognosis of such atypical ALL cases.

摘要

历史上,伴有颗粒性原始细胞的急性淋巴细胞白血病(ALL)被称为“颗粒性ALL”,但在当前的世界卫生组织(WHO)分类中,这并非一个独立的实体。这种罕见的形态学特征主要与B细胞免疫表型相关,而具有显著细胞质颗粒的T-ALL极为罕见,可用的遗传、分子和预后数据有限。在此,我们报告一例9岁女孩患ALL的独特病例。初始血液检查显示白细胞增多,外周血和骨髓形态均显示大量具有颗粒状细胞质和双叶核的原始细胞。免疫表型分析证实为T细胞起源。该患者KMT2A::AFDN检测呈阳性,伴有WT1过表达,通过二代测序(NGS)检测到NRAS和EZH2突变。值得注意的是,该患者治疗后实现完全缓解,近四年无复发迹象。她对传统治疗的良好反应凸显了分子表型分析在该疾病治疗中的重要性。持续使用NGS收集相关分子数据对于进一步了解此类非典型ALL病例的分子表型和预后至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3547/11582310/dbfc151f1024/12672_2024_1600_Fig1_HTML.jpg

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