摘要
目的 探讨毛细胞白血病(HCL)的临床表现及诊治效果。方法 回顾性分析2010年10月—2018年10月本院血液科收治的首次诊断为HCL的20例患者临床资料,分析临床症状及相关诊治资料。结果 本组病例以男性居多,占到65%;中位年龄51.6岁,其中18例经典型HCL,占比90.0%,2例变异型HCL,占比10%;首发症状主要是腹胀、面色苍白、乏力,其次是出血,个别患者伴B症状;临床病理表现主要是脾肿大、血小板减少,其次是肝肿大、淋巴结肿大、全血细胞减少,再次是变异型白细胞计数增高。病理活检表明所有病例均表现为增生活跃至极度活跃。通过流式细胞学分析,毛细胞表达CDl9、CD20、CD22、CD25、CDl03、CDllc;经典型HCL部分病例出现BRAFV600E突变;大部分患者携带乙肝病毒;核苷类似物是HCL治疗有效药物,对变异型HCL联合用药治疗有确切效果,本组临床治疗有效率达到80%。结论 HCL以男性多见,临床表现多样,以脾肿大、血小板减少为主,免疫表型特异性表达多样,核苷类似物为主要有效治疗药物,对变异型HCL联合治疗有良好效果。
Objective To explore the clinical manifestation, diagnosis and treatment efficacy of hairy cell leukemia(HCL).Methods The clinical data of 20 HCL patients those were diagnosed for the first time in the hematology department of union hospital affiliated to Fujian Medical University from October 2010 to October 2018 were retrospectively analyzed, and clinical symptoms and related diagnosis and treatment data were analyzed.Results Among the patients, the majority of cases were male, accounting for 65%. The median age was 51.6 years. There were 18 cases with typical HCL(90.0%) and 2 cases with variant HCL(10%). The initial symptoms were abdominal distension, pallor and fatigue, followed by bleeding, and some patients were accompanied by symptom B. The main clinicopathological manifestations were splenomegaly and thrombocytopenia, followed by hepatomegaly, lymphadenopathy, pancytopenia, and increased variant white blood cell count. Pathological biopsy indicated that all cases showed hyperactive to hyperactive. By flow cytometry analysis, hair cells expressed CDl9, CD20, CD22, CD25, CDl03 and CDllc, and BRAFV600 E mutation was found in typical hair cell leukemia. Most of the patients had hepatitis B virus infection;Nucleoside analogues are effective drugs for the treatment of HCL, and combined treatment have a definite effect on the variant HCL. The clinical effective rate of this group is 80%.Conclusions HCL is more common in males and has various clinical manifestations, which were mainly splenomegasis and thrombocytopenia. Specific expression of immunophenotype is diverse. Nucleoside analoges are the main effective drugs for HCL, and the combined treatment of variant HCL could achieve good effect.
作者
徐淑娟
杨百华
李鑫
张淑遐
翁萍
王怡婷
耿海丽
陈萍
Xu Shujuan;Yang Baihua;Li Xin;Zhang Shuxia;Weng Ping;Wang Yiting;Geng Haili;Chen Ping(Fujian insitute of hematology&Fujian provincial ley laboratory on hematology&Fujian Medical University Union hospital;Department of radiation oncology,Fujian cancer hospital&Cancer hospital of Fujian Medical University,Fuzhou,Fujian,350014,China)
出处
《齐齐哈尔医学院学报》
2022年第14期1328-1331,共4页
Journal of Qiqihar Medical University
基金
2019年福建省中青年教师教育科研项目(JAT190187)
福建省血液医学中心建设项目[闽政办(2017)4号]
国家及省临床重点专科资助。
关键词
毛细胞白血病
免疫表型
临床表现
核苷类似物
Hair cell leukemia
Immunophenotype
Clinical manifestations
Nucleoside analogues