PDF(Pubmed)
Abstract：
OBJECTIVE: 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.
METHODS: 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.
RESULTS: 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 BRAFV600E 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 (P=0.01), and there was no significant difference in the impact of different treatment regiments on the OS of HCL patients (P=0.328).
CONCLUSIONS: 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.
METHODS: 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.
RESULTS: 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 BRAFV600E 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 (P=0.01), and there was no significant difference in the impact of different treatment regiments on the OS of HCL patients (P=0.328).
CONCLUSIONS: 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.
摘要：
目的：分析临床特点，罕见毛细胞白血病（HCL）的诊断，治疗和预后，以期为HCL的临床和基础研究提供新的参考。
方法：收集福建医科大学附属协和医院收治的17例HCL患者的临床资料,收集2016年1月1日至2023年7月1日莆田学院附属医院和赣南医学院第一附属医院,和临床特征，分析HCL患者的诊治效果及预后。采用Kaplan-Meier法进行生存分析。同时,检索PubMed的最新文献，系统地讨论HCL的诊断和治疗研究进展。
结果：在这项研究中，有11名男性和6名女性，诊断时的中位年龄为59.5（30-81）岁，从临床症状或体征出现到诊断的中位时间为4.5（0.5-28.5）个月。有9例(52.94%)有淋巴瘤B症状(发热,晚上出汗，和体重减轻），15例(88.24%)伴有脾肿大(3例轻度脾肿大,中度脾肿大4例，和8例巨脾肿大)，BRAFV600E突变阳性率为76.47%(13/17)。这项研究中的所有患者都接受了治疗，其中11例接受克拉屈滨治疗，3与干扰素，2采用FC方案，1采用R-CVP方案+克拉屈滨。中位随访时间为39(范围，2-83）个月，3名患者死亡,都是由于疾病进展导致化疗失败。HCL-v患者的预后明显差于cHCL患者(P=0.01),不同治疗方案对HCL患者OS的影响差异无统计学意义(P=0.328)。
结论：HCL是一种罕见的临床惰性血液肿瘤，对克拉屈滨很敏感,随着新型分子靶向药物和免疫治疗等精准治疗的出现，在HCL的临床实践中也发挥着不可或缺的作用。
方法：收集福建医科大学附属协和医院收治的17例HCL患者的临床资料,收集2016年1月1日至2023年7月1日莆田学院附属医院和赣南医学院第一附属医院,和临床特征，分析HCL患者的诊治效果及预后。采用Kaplan-Meier法进行生存分析。同时,检索PubMed的最新文献，系统地讨论HCL的诊断和治疗研究进展。
结果：在这项研究中，有11名男性和6名女性，诊断时的中位年龄为59.5（30-81）岁，从临床症状或体征出现到诊断的中位时间为4.5（0.5-28.5）个月。有9例(52.94%)有淋巴瘤B症状(发热,晚上出汗，和体重减轻），15例(88.24%)伴有脾肿大(3例轻度脾肿大,中度脾肿大4例，和8例巨脾肿大)，BRAFV600E突变阳性率为76.47%(13/17)。这项研究中的所有患者都接受了治疗，其中11例接受克拉屈滨治疗，3与干扰素，2采用FC方案，1采用R-CVP方案+克拉屈滨。中位随访时间为39(范围，2-83）个月，3名患者死亡,都是由于疾病进展导致化疗失败。HCL-v患者的预后明显差于cHCL患者(P=0.01),不同治疗方案对HCL患者OS的影响差异无统计学意义(P=0.328)。
结论：HCL是一种罕见的临床惰性血液肿瘤，对克拉屈滨很敏感,随着新型分子靶向药物和免疫治疗等精准治疗的出现，在HCL的临床实践中也发挥着不可或缺的作用。
