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21例急性淋巴细胞白血病合并中枢神经系统白血病的临床特征与预后

[Clinical Characteristics and Prognosis of 21 Cases of Acute Lymphoblastic Leukemia with Central Nervous System Leukemia].

作者信息

Jin Xiang-Shu, Yang Lan, Wang Bian-Hong, Li Hong-Hua, Yu Li, Jing Yu

机构信息

Department of Hematology,Chinese PLA General Hospital,Beqing 100853, China.

Department of Hematology,Chinese PLA General Hospital,Beqing 100853, China. E-mail:

出版信息

Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2016 Dec;24(6):1675-1682. doi: 10.7534/j.issn.1009-2137.2016.06.012.

Abstract

OBJECTIVE

To investigate the clinical characteristics, therapeutic efficacy survival and prognosis of patients with adult acute lymphoblastic leukemia (ALL) accompanied by central nervous system leukemia (CNSL).

METHODS

The clinical and cerebrospinal fluid (CSF) features, diagnosis and treatment, therapeutic efficacy and survival rate of 21 cases of acute lymphoblastic leukemia (ALL) with central nervous system involvement (CNSL) were analyzed retrospectively.

RESULTS

Out of 21 cases, 10 cases were B cell acute lymphoblastic leukemia(B-ALL), 6 cases were T cells acute lymphoblastic(T-ALL), 4 cases were determined as no clear typing, 1 case was Burkitt lymphoma/leukemia, 7 patients had CNSL at the time of diagnosis, and 14 patients were showed CNS relapse. Clinical manifestations included headache, facial paralysis, limb weakness and blurred vision, etc. Their median follow-up time was 19(6-40) months,from them 10 cases died, 7 cases survived, 4 cases were lost to follow up. Patients had CNSL at the time of diagnosis, their peripheral blood LDH≥600 U/L or not achieving complete remission (CR) after 1 course of treatment with poor prognosis, and the difference is significant (P< 0.05). Radiotherapy and allogeneic stem cell transplantation (allo-HSCT) could improve the patient's survival. Multivariate analysis showed that the LDH and allo-HSCT was significantly correlated with survival time (P=0.048, P=0.013).

CONCLUSION

There are no specific clinical manifestations, CSF features and imaging manifestations of ALL accompanied by CNSL, and the diagnosis of CSF is needed to find the leukemia cells in CSF. The factors for poor prognosis include LDH≥600 U/L, no CR of patients after 1 course of treatment, existence of CNSL at the diagnosis. ALL patients with CNSL have a poor prognosis. Intrathecal injection combined with systemic chemotherapy, radiation therapy and allo-HSCT after CR is the feasible and effective treatment regimen.

摘要

目的

探讨成人急性淋巴细胞白血病(ALL)合并中枢神经系统白血病(CNSL)患者的临床特征、治疗疗效、生存及预后情况。

方法

回顾性分析21例合并中枢神经系统受累(CNSL)的急性淋巴细胞白血病(ALL)患者的临床及脑脊液(CSF)特点、诊断与治疗、治疗疗效及生存率。

结果

21例患者中,B细胞急性淋巴细胞白血病(B-ALL)10例,T细胞急性淋巴细胞白血病(T-ALL)6例,4例分型不明确,1例为伯基特淋巴瘤/白血病,7例诊断时即有CNSL,14例出现CNS复发。临床表现包括头痛、面瘫、肢体无力、视物模糊等。中位随访时间为19(6 - 40)个月,其中死亡10例,存活7例,失访4例。诊断时即有CNSL的患者,外周血乳酸脱氢酶(LDH)≥600 U/L或1个疗程治疗后未达完全缓解(CR)者预后差,差异有统计学意义(P<0.05)。放疗及异基因造血干细胞移植(allo-HSCT)可提高患者生存率。多因素分析显示,LDH及allo-HSCT与生存时间显著相关(P = 0.048,P = 0.013)。

结论

ALL合并CNSL无特异性临床表现、CSF特点及影像学表现,需行CSF检查查找脑脊液中的白血病细胞。预后不良因素包括LDH≥600 U/L、1个疗程治疗后未达CR、诊断时存在CNSL。ALL合并CNSL患者预后差。CR后鞘内注射联合全身化疗、放疗及allo-HSCT是可行有效的治疗方案。

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