文章摘要
李雪雪,许倩文,王兴兵.中枢神经系统白血病临床特征、复发及预后因素分析[J].安徽医药,2025,29(5):920-926.
中枢神经系统白血病临床特征、复发及预后因素分析
Analysis of clinical features, factors associated with relapse and prognosis of patients with central nervous system leukemia
  
DOI:10.3969/j.issn.1009-6469.2025.05.013
中文关键词: 急性白血病  中枢神经系统白血病  异基因造血干细胞移植  鞘内注射  影响因素
英文关键词: Acute leukemia  Central nervous system leukemia  Allogeneic hematopoietic stem cell transplantation  Intrathecal injection  Influencing factors
基金项目:
作者单位E-mail
李雪雪 安徽医科大学省立临床学院安徽合肥 230001
中国科学技术大学附属第一医院血液科安徽合肥 230001 
 
许倩文 中国科学技术大学附属第一医院血液科安徽合肥 230001  
王兴兵 安徽医科大学省立临床学院安徽合肥 230001
中国科学技术大学附属第一医院血液科安徽合肥 230001 
wangxingbing@ustc.edu.cn 
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中文摘要:
      目的探讨中枢神经系统白血病( CNSL)的临床特征、复发及预后因素。方法回顾性分析 2015年 6月至 2022年 12月中国科学技术大学附属第一医院收治的急性白血病合并 CNSL病人临床资料,探究其临床特征、复发情况及预后相关因素。结果共纳入 101例急性白血病合并 CNSL病人,其中急性淋巴细胞白血病( ALL)病人 73例( 72.3%)急性髓系白血病( AML)病人 28例( 27.7%)。 24(23.8%)例病人 CNSL发生于初诊时, 46例( 59.7%)发生于骨髓缓解期, 5例( 6.5,%)发生于骨髓未缓解期, 26例( 33.8%)发生于骨髓复发期。 94例( 96.9%)病人在接受 CNSL相关治疗后达到中枢神经系统完全缓解( CR)38例(40.4%)病人在后续随访中出现 2次或多次 CNSL复发(其中 ALL29例, AML9例)。鞘内注射( IT)化疗联合异基因造血干细胞,移植( allo-HSCT)者的复发率低于未联合 allo-HSCT者( 19.0%比 43.8%,P=0.039)且 IT化疗联合 allo-HSCT者中位无病生存期(DFS)(30±3.9)个月显著优于未联合 allo-HSCT者( 11.0±2.9)个月( P=0.015)。在初次,诊断时,白细胞计数大于 50×109/L亦是影响 CNSL复发的高危因素( P=0.040)。对于 ALL合并 CNSL病人,初诊时高白细胞水平是病人 DFS的危险因素( P=0.009)。 101例病人的中位生存时间( mOS)为 6.0(1.0,96.0)个月,联合 allo-HSCT治疗者与单独化疗及放疗相比能获得更好的中位 OS[(40.0±20.2)个月比(12.0±3.7)个月, P=0.041]。大于等于 6次规范的 IT化疗,亦可改善病人的中位 OS[(30.0±8.7)个月比(7.0±2.6)个月, P=0.002]。针对 ALL合并 CNSL病人,联合 HD-MTX化疗的病人相对于未联合 HD-MTX者 2年 OS率更高[( 64.9±11.7)%比( 34.3±10.2)%,P=0.029]。结论对于急性白血病合并 CNSL的病人,初诊时白细胞 >50×109/L是影响其复发的高危因素。 IT化疗基础上联合 allo-HSCT能降低 CNSL复发率、改善 DFS及 OS。大于等于 6次规范的 IT化疗,亦可改善病人的中位 OS。对于 ALL病人,初诊时白细胞增高是影响 CNSL病人 DFS的独立危险因素,选择 HD-MTX方案化疗能延长 CNSL病人的 OS。
英文摘要:
      Objective To investigate the clinical features, factors associated with relapse and prognosis of patients with central ner-vous system leukemia (CNSL). Method We retrospectively analyzed the clinical data of acute leukemia patients with CNSL at theFirst Affiliated Hospital of University of Science and Technology of China from June 2015 to December 2022 to investigate the clinicalfeatures, factors related to relapse and prognosis.Results Data of 101 patients with CNSL were collected and analyzed, including 73(72.3%) cases of acute lymphoblastic leukemia (ALL) and 28 (27.7%) cases of acute myeloid leukemia (AML). CNS involvement oc-curred in 24 (23.8%) patients at the time of the initial leukemia diagnosis, in 46 (59.7%) at the state of bone marrow remission, in five(6.5%) at bone marrow non-remission, and in 26(33.8%) at the state of relapse. Nighty-four (96.9%) patients achived CNS-CR after CNS-directed treatment, of whom 38 (40.4%) had subsequent second or multiple CNS relapse (ALL, n=29; AML, n=9). The rate of CNSrelapse in patients treated with intrathecal (IT) chemotherapy in combination with allogeneic hematopoietic stem cell transplantation (al.lo-HSCT) was lower than those without allo-HSCT (19.0% vs. 43.8%,P=0.039), and the disease-free survival (DFS) of CNSL patients treated with IT chemotherapy combined with allo-HSCT (30.0±3.9) months was significantly superior than those without allo-HSCT (11.0±2.9) months (P=0.015). The leukocyte level greater than 50×109/L was also a high-risk factor for CNSL recrudesce. The high leu-kocyte level at initial diagnosis is a risk factor for DFS of the ALL patients with CNSL (P=0.009). The median overall survival (mOS) of the 101 patients was 6.0 (1.0-96.0) months, chemotherapy or cranial radiation in combination with allo-HSCT resulted in better median OS compared with chemotherapy and cranial radiation alone [(40.0±20.2)months vs. (12.0±3.7)months, P=0.041]. Patients with more than or equal to six cycles of standard IT chemotherapy showed longer median OS [(30.0±8.7)months vs.(7.0±2.6)months,P=0.002]. The ALL patients with CNSL treated with high-dose methotrexate-based (HD-MTX) chemotherapy had higher 2-year OS rates than those without HD-MTX chemotherapy [(64.9±11.7)% vs. (34.3±10.2)%,P=0.029].Conclusions For patients with acute leukemia complicat-ed with CNSL, leukocyte level greater than 50×109/L is a high-risk factor for CNSL recrudesce. IT chemotherapy combined with allo-HSCT can reduce the recurrence rate of CNSL and also improve DFS and OS in patients. Patients with more than or equal to six cyclesof standard IT chemotherapy show longer median OS. For the ALL patients with CNSL, high leukocyte level at initial diagnosis is an in-dependent risk factor for DFS, and HD-MTX chemotherapy induces prolonged OS than those without HD-MTX chemotherapy.
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