| 摘要: |
| 目的 分析多毛细胞白血病（HCL）的临床特征、诊疗及预后。方法 收集整理2016年1月至2021年2月福建医科大学附属协和医院收治的11例HCL患者的临床资料，分析HCL的临床特征、诊治疗效及预后情况。结果 11例HCL患者中男7例、女4例，确诊时中位年龄为57（30～81）岁，从出现临床症状或体征到诊断的中位时间为5.0（0.5～26.0）个月。诊断时伴淋巴瘤B症状（即发热、盗汗、体重减低）者有6例，伴有感染者7例（均为肺部感染），淋巴结肿大3例；所有患者均伴有脾肿大（轻度脾大3例，中度脾大2例，巨脾6例）。11例患者均行一代或二代基因测序检测，B-Raf原癌基因丝氨酸/苏氨酸蛋白激酶V600E突变型（BRAFV600E）突变阳性率为81.82%（9/11）；外周血常规示两系减少4例（36.36%），三系减少7例（63.64%），单核细胞减少9例（81.82%）。11例患者中，除1例采用FC方案（氟达拉滨50 mg第1～3天；环磷酰胺0.3 g第1～3天）及1例第1个疗程采用R-CVP方案（利妥昔单抗500 mg第1天；环磷酰胺1.1 g第2天；长春地辛4 mg第2天；地塞米松15 mg第1～5天）后改为克拉屈滨以外，其余9例患者均接受克拉屈滨单药治疗。1例采用FC方案治疗的患者1个疗程后即获得完全缓解，余10例中克拉屈滨单疗程治疗者有7例、2个疗程治疗者有3例，4例在克拉屈滨治疗后1～3个月疗效评估为部分缓解，6例4～12个月治疗疗效评估达完全缓解，对克拉屈滨治疗总体反应率为100.00%（10/10）。10例采用克拉屈滨治疗的患者在治疗后出现2～4级血液学不良反应，其中7例（63.64%，7/11）发生绝对中性粒细胞计数减少，6例（54.55%，6/11）患者发生血小板计数减少，4例（36.36%，4/11）发生中性粒细胞缺乏伴发热，3例（27.27%，3/11）发生肺部感染或上呼吸道感染，伴随感染症状患者经抗感染及支持治疗后好转。除血液学不良反应外，无其他不良反应发生。11例HCL患者中位随访时间为14（3～61）个月，中位总生存期与无进展生存期均未达到。随访期间，无患者发生HCL疾病进展、复发或死亡。结论 HCL为临床罕见的惰性血液肿瘤，其对克拉屈滨敏感且不良反应可控，对于复发、难治HCL患者可选择进入临床试验或考虑联用其他靶向抑制剂或单克隆抗体。 |
| 关键词: 多毛细胞白血病 诊断 鉴别诊断 化学治疗 克拉屈滨 预后 |
| DOI：10.16781/j.CN31-2187/R.20210719 |
| 投稿时间：2021-07-23修订日期：2021-11-07 |
| 基金项目:国家自然科学基金面上项目（82073582），广东省教育厅高校特色创新项目（2020KTSCX048），广东医科大学学科建设项目（4SG22003G，4SG21209G），广东医科大学科技创新专项计划项目（4SG22046G）. |
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| Clinical features, diagnosis and treatment of hairy cell leukemia |
| LIU Yan-quan1，2,YIN Yue2,ZENG Min-juan3,YANG Hui1,CHEN Yu-ting1,TANG Huan-wen1* |
(1. Department of Hematology, The First Clinical Medical College of Guangdong Medical University, Dongguan 523808, Guangdong, China;
2. Fujian Institute of Hematology, Department of Hematology, Fujian Medical University Union Hospital, Fuzhou 350001, Fujian, China;
3. Department of Anatomy, School of Basic Medicine, Guangdong Medical University, Dongguan 523808, Guangdong, China
*Corresponding author) |
| Abstract: |
| Objective To analyze the clinical features, diagnosis and treatment and prognosis of hairy cell leukemia (HCL). Methods The clinical data of 11 HCL patients admitted to Fujian Medical University Union Hospital from Jan. 2016 to Feb. 2021 were collected and sorted out, and the clinical features, diagnosis and treatment effects and prognosis of HCL were analyzed. Results There were 7 males and 4 females, the median age at diagnosis was 57 (30-81) years old, and the median time from the onset of clinical symptoms or signs to diagnosis was 5.0 (0.5-26.0) months. At diagnosis, there were 6 cases with lymphoma B symptoms (fever, night sweating, and weight loss), 7 cases with infection (all pulmonary infection), and 3 cases with lymphadenopathy; all cases were accompanied by splenomegaly (3 cases of mild splenomegaly, 2 cases of moderate splenomegaly, and 6 cases of megasplenomegaly). All the 11 patients underwent first-or second-generation sequencing. The positive rate of B-Raf proto-oncogene, serine/threonine kinase V600E mutant type (BRAFV600E) mutation was 81.82% (9/11); peripheral blood routine showed that the two lines decreased in 4 (36.36%) cases, the three lines decreased in 7 (63.64%) cases, and the monocyte decreased in 9 (81.82%) cases. Among the 11 patients, except 1 patient who received FC regimen (fludarabine 50 mg and cyclophosphamide 0.3 g for the first 3 d) and 1 patient who received R-CVP regimen (rituximab 500 mg for the first day; cyclophosphamide 1.1 g for the second day; vindesine 4 mg for the second day; dexamethasone 15 mg for the first 5 d) in the first course of treatment and changed to cladribine in the next course, the remaining 9 patients received cladribine monotherapy. One patient obtained complete remission after 1 course of treatment with FC regimen. Among the remaining 10 patients, 7 patients were treated with single course of cladribine and 3 patients were treated with 2 courses. Among them, 4 patients were treated with cladribine for 1-3 months, the curative effect was evaluated as partial remission, and the remaining 6 patients reached complete remission after 4-12 months of treatment. The overall response rate of 10 patients to cladribine was 100.00%. Ten of the 11 patients had adverse hematological reactions of grade 2-4 after cladribine treatment: 7 (63.64%, 7/11) cases had neutropenia, 6 (54.55%, 6/11) cases had thrombocytopenia, 4 (36.36%, 4/11) cases developed agranulocytosis with fever, and 3 (27.27%, 3/11) cases developed lung infection or upper respiratory tract infection. The patients with infection symptoms improved after anti-infection and supportive treatment. There were no other adverse reactions except hematological adverse reactions. The median follow-up time of 11 HCL patients was 14 (3-61) months, and the median overall survival and progression-free survival were not reached. During the followed-up, no patients had HCL disease progression, recurrence, or death. Conclusion HCL is a rare clinically indolent hematological tumor, which is sensitive to cladribine and has controllable adverse reactions. Patients with relapsed or refractory HCL can choose to enter clinical trials or consider joint use of other targeted inhibitors or monoclonal antibodies. |
| Key words: hairy cell leukemia diagnosis differential diagnosis chemotherapy cladribine prognosis |
