Huang He, Liu Yanquan, Zeng Minjuan, Chen Xiaojun, Yin Yue, Guo Huidong, Yan Zhimin, Lin Jie
Department of Cardiovascular Medicine (Cardiology), The First Affiliated Hospital of Gannan Medical University Ganzhou 341000, Jiangxi, China.
Department of Hematology, The First Dongguan Affiliated Hospital of Guangdong Medical University Dongguan 523808, Guangdong, China.
Am J Transl Res. 2024 Feb 15;16(2):466-476. doi: 10.62347/WFQM3371. eCollection 2024.
To analyze the clinical features, diagnosis and treatment and prognosis of the rare hairy cell leukemia (HCL), in order to provide new references for the clinical and basic research of HCL.
The clinical data of 17 patients with HCL admitted to Fujian Medical University Union Hospital, the Affiliated Hospital of Putian University and the First Affiliated Hospital of Gannan Medical University from January 1, 2016 to July 1, 2023 were collected and retrospectively studied, and the clinical features, diagnosis and treatment effects and prognosis of patients with HCL were analyzed. The Kaplan-Meier method was used for survival analysis. Meanwhile, the latest literature from PubMed was retrieved to systematically discuss the research progress in the diagnosis and treatment of HCL.
In this study, there were 11 males and 6 females, the median age at diagnosis was 59.5 (30-81) years old, and the median time from the onset of clinical symptoms or signs to diagnosis was 4.5 (0.5-28.5) months. There were 9 cases (52.94%) with lymphoma B symptoms (fever, night sweating, and weight loss), 15 cases (88.24%) were accompanied by splenomegaly (3 cases of mild splenomegaly, 4 cases of moderate splenomegaly, and 8 cases of megasplenomegaly), the positive rate of mutation is 76.47% (13/17). All patients in this study were treated, of which 11 were treated with Cladribine, 3 with Interferon, 2 with FC regimen, and 1 with R-CVP regimen + Cladribine. The median follow-up time was 39 (range, 2-83) months, 3 patients died, all due to failure of chemotherapy due to disease progression. The prognosis of HCL-v patients was significantly worse than that of cHCL patients (=0.01), and there was no significant difference in the impact of different treatment regiments on the OS of HCL patients (=0.328).
HCL is a rare clinically indolent hematological tumor, which is sensitive to Cladribine, with the emergence of precision treatments such as the novel molecular-targeted drugs and immunotherapy also plays an indispensable role in clinical practice of HCL.
分析罕见的毛细胞白血病（HCL）的临床特征、诊断、治疗及预后，为HCL的临床及基础研究提供新的参考依据。
收集福建医科大学附属协和医院、莆田学院附属医院及赣南医学院第一附属医院2016年1月1日至2023年7月1日收治的17例HCL患者的临床资料，进行回顾性研究，分析HCL患者的临床特征、诊断、治疗效果及预后。采用Kaplan-Meier法进行生存分析。同时检索PubMed最新文献，系统探讨HCL诊断与治疗的研究进展。
本研究中，男性11例，女性6例，诊断时中位年龄为59.5（30 - 81）岁，从出现临床症状或体征到诊断的中位时间为4.5（0.5 - 28.5）个月。有9例（52.94%）出现淋巴瘤B症状（发热、盗汗、体重减轻），15例（88.24%）伴有脾肿大（轻度脾肿大3例，中度脾肿大4例，巨脾8例）， 突变阳性率为76.47%（13/17）。本研究所有患者均接受了治疗，其中11例接受克拉屈滨治疗，3例接受干扰素治疗，2例接受FC方案治疗，1例接受R-CVP方案 + 克拉屈滨治疗。中位随访时间为39（范围2 - 83）个月，3例患者死亡，均因疾病进展化疗失败。HCL-v患者的预后明显差于cHCL患者（ = 0.01），不同治疗方案对HCL患者总生存期的影响无显著差异（ = 0.328）。
HCL是一种临床罕见的惰性血液肿瘤，对克拉屈滨敏感，新型分子靶向药物和免疫治疗等精准治疗的出现也在HCL的临床实践中发挥着不可或缺的作用。
