Sun Yingxin, Wang Qinrong, Zhang Zhiyu, Wang Qian, Cen Jiannong, Zhu Mingqing, Pan Jinlan, Liu Dandan, Shen Hongjie, Cai Yifeng, Chen Suning
Affiliated Hospital of Nantong University, Nantong, China.
The First Affiliated Hospital of Soochow University, National Clinical Research Center for Hematologic Diseases, Jiangsu Institute of Hematology, Suzhou, China.
Ann Hematol. 2024 Dec;103(12):5325-5332. doi: 10.1007/s00277-024-06032-z. Epub 2024 Oct 8.
The classification of atypical chronic myeloid leukemia (aCML) and chronic neutrophilic leukemia (CNL) as a single disease entity remains a topic of debate. To elucidate the characteristics of both entities, this retrospective cohort study was conducted, encompassing 36 cases of aCML and 18 cases of CNL. We discovered that aCML and CNL presented distinct blood counts, genetics, molecular profiles and outcomes. Specifically, hemoglobin levels (P < 0.001) and platelet counts (P < 0.001) were significantly lower in aCML cases than in CNL cases, with no significant difference in mean white blood cells (P = 0.637). The proportion of abnormal karyotypes was higher in aCML cases compared with CNL cases (P = 0.010). Notably, we found that aCML and CNL showed distinct gene expression profiles by transcriptome sequencing technology. The median follow-up duration for the entire cohort was 8 months (rang 0.4 to 36.6 months), and the median overall survival (OS) was significantly shorter in aCML cases (7.3 months, 95%CI 5.4 to 20.5 months) than in CNL cases (median OS not reached). The one-year OS rate for aCML patients was 31.0% (9/29), compared to 92.9% (13/14) for CNL patients. In conclusion, our study supports the notion that aCML and CNL are indeed distinct disease entities characterized by unique hematological features and clinical outcomes.
将非典型慢性髓系白血病（aCML）和慢性嗜中性粒细胞白血病（CNL）归为单一疾病实体仍存在争议。为阐明这两种疾病实体的特征，我们开展了这项回顾性队列研究，纳入了36例aCML患者和18例CNL患者。我们发现，aCML和CNL在血细胞计数、遗传学、分子特征及预后方面表现各异。具体而言，aCML患者的血红蛋白水平（P < 0.001）和血小板计数（P < 0.001）显著低于CNL患者，而平均白细胞计数无显著差异（P = 0.637）。与CNL患者相比，aCML患者中异常核型的比例更高（P = 0.010）。值得注意的是，通过转录组测序技术，我们发现aCML和CNL呈现出不同的基因表达谱。整个队列的中位随访时间为8个月（范围0.4至36.6个月），aCML患者的中位总生存期（OS）显著短于CNL患者（7.3个月，95%CI 5.4至20.5个月）（CNL患者的中位OS未达到）。aCML患者的一年总生存率为 31.0%（9/29），而CNL患者为92.9%（13/14）。总之，我们的研究支持以下观点：aCML和CNL确实是不同的疾病实体，具有独特的血液学特征和临床预后。
