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慢性髓性白血病的爆发期呈现为早期T细胞前体急性淋巴细胞白血病

Shuyu E¹, Jie Xu¹, Sa A Wang¹

¹Department of Hematopathology, The University of Texas M.D. Anderson Cancer Center, Houston, TX, US.

American journal of clinical pathology

|September 5, 2024

概括

慢性髓性白血病爆发阶段 (CML-BP) 很少会出现早期T细胞前体急性淋巴细胞白血病 (ETP-ALL). 这些侵袭性病例需要密集治疗,干细胞移植可能会改善结果.

科学领域:

血液学血液学血液学
在瘤学瘤学.
分子生物学分子生物学

背景情况:

慢性骨髓性白血病爆发期 (CML-BP) 通常涉及骨髓性或前体B免疫类型.
早期T细胞前体急性淋巴细胞白血病 (ETP-ALL) 是一种罕见的急性淋巴细胞白血病亚型.
带有ETP-ALL的CML-BP异常罕见,这给诊断和治疗带来了挑战.

研究的目的:

描述CML-BP和ETP-ALL患者的临床病理学,免疫类型和分子遗传特征.
为了审查有关这种罕见实体的文献.
评估治疗结果和潜在的治疗策略.

主要方法:

3名患有CML-BP和ETP-ALL的患者的病例系列.
针对BCR::ABL1重新排列的免疫类型和光在位杂交 (FISH) 测定.
下一代测序用于突变分析.
文献综述. 这是一个文献综述.

主要成果:

一名患者有慢性髓性白血病慢性阶段的病史;另外两名患者被直接诊断出.
在两个案例中,BCR::ABL1重新排列证实了CML-BP.
爆发对细胞质CD3,CD7,CD33,CD117和色CD5呈阳性,对CD1a和CD8呈阴性.
发现的突变包括TET2,BCOR,RUNX1和STAG2.
两名患者在诊断后大约40天内死亡,尽管接受了化疗和氨酸激酶抑制剂.
一名患者在干细胞移植后在诊断后的14个月内存活.

结论:

CML-BP可以呈现为ETP-ALL,一种罕见但具有攻击性的临床实体.
强化治疗,包括化疗和氨酸激酶抑制剂,是适用的.
干细胞移植可能为特定患者提供生存益处.

关键词:

在CML中,CML是CML.在 ETP-ALL 中,所有爆炸阶段的爆发阶段

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