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中国循证儿科杂志

中国循证儿科杂志 ›› 2022, Vol. 17 ›› Issue (4): 296-301.DOI: 10.3969/j.issn.1673-5501.2022.04.006

• 论著 • 上一篇    下一篇

以周围性面瘫为首发症状的急性白血病患儿16例病例系列报告

刘京1,陆爱东1,左英熹1,吴珺2,黄志卓3,贾月萍1,丁明明1,张乐萍1,秦炯1   

  1. １ 北京大学人民医院儿科  北京，100044；２ 北京大学首钢医院儿科  北京，100144 
  • 收稿日期:2022-04-09 修回日期:2022-05-26 出版日期:2022-08-25 发布日期:2022-09-28
  • 通讯作者: 张乐萍，秦炯
  • 基金资助:
    北京市卫生健康委员会北京市临床重点专科项目（2018）-儿科：京卫医-2018204

16 cases of acute leukemia in children with peripheral facial paralysis as the initial symptom: Ａ case series report

LIU Jing1, LU Aidong1, ZUO Yingxi1, WU Jun2, HUANG Zhizhuo1, JIA Yueping1, DING Mingming1, ZHANG Leping1, QIN Jiong1   

  1. 1 Department of Pediatrics, Peking University People's Hospital, Beijing 100044, China; 2 Department of Pediatrics, Peking University Shougang Hospital, Beijing 100144, China
  • Received:2022-04-09 Revised:2022-05-26 Online:2022-08-25 Published:2022-09-28
  • Contact: ZHANG Leping, QIN Jiong

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摘要： 背景：以周围性面瘫起病的儿童急性白血病国内外相关报道较少，临床易被误诊。 目的：探讨以周围性面瘫为首发症状的急性白血病患儿的临床特点、治疗和预后。 设计：病例系列报告。 方法：纳入2010年1月至2021年9月北京大学人民医院儿科收治的以周围性面瘫为首发症状的急性白血病患儿，分析其临床特征、辅助检查、治疗和预后。 主要结局指标：面瘫结局以及5年无事件生存（EFS）、总生存（OS）。 结果：研究期间共收治急性白血病患儿1 018例，其中ALL 728例，AML 290例。16例（1.6%）患儿以周围性面瘫为首发症状起病，其中男7例、女9例，中位年龄5（2~14）岁，从面瘫起病至确诊白血病中位时间 9.5（1~31）d；其中ALL B细胞型2例（12.5%），ALL T细胞型1例（6.2%），AML M2型13例（81.3%）。16例均为单侧面瘫，左侧5例（31.2%），右侧11例（68.8%）。15例行头颅MR检查，2例示硬脑膜、软脑膜增厚强化（其中1例伴面神经可疑损伤），1例示眼内直肌及视神经眶内段白血病浸润，3例示乳突炎。15例行脑脊液检查，其中1例脑脊液残留阳性，1例曾出现脑脊液蛋白升高。1例确诊白血病后放弃治疗，余15例予以化疗。8例规律化疗后骨髓持续缓解，6例行造血干细胞移植，1例化疗期间因合并多脏器功能不全而停止化疗，复发后死亡。13例在1个月内面瘫症状消失，2例在3个月内面瘫症状消失。中位随访时间44.5（0.23~111）月，5年EFS为（48.2±13.0）％，5年OS为（87.5±8.3）％。13例AML患儿5年EFS为(51.3±14.6）%，5年OS为（84.6±10.0）%，其中造血干细胞移植患儿5年OS为100%，仅化疗患儿5年 OS为（87.5±11.7）%，差异无统计学意义（P=0.48）。 结论：以周围性面瘫为首发症状的急性白血病患儿早期易被误诊。对于儿童周围性面瘫，在类固醇激素治疗前应排除继发性病因。以面瘫起病的急性白血病类型以AML为主，需考虑强化中枢神经系统治疗。周围性面瘫起病的AML患儿总体生存率无明显影响，但更易复发。

关键词: 周围性面瘫, 儿童, 白血病

Abstract: Background：At present, there are few reports about acute leukemia in children with peripheral facial paralysis as the initial symptom, and it is easy to be misdiagnosed clinically. Objective：To investigate the clinical characteristics，treatment and prognosis of children diagnosed with acute leukemia presented with peripheral facial paralysis as the initial symptom. Design：Case series report. Methods：The children diagnosed with acute leukemia presented with peripheral facial paralysis as the initial symptom admitted to the Department of Pediatrics of Peking University People's Hospital from January 2010 to September 2021 were included. The clinical characteristics, laboratory examinations, treatment and outcome were analyzed. Main outcome measures：The outcome of facial paralysis，5year eventfree survival（EFS），overall survival（OS）. Results：A total of 1 018 children with acute leukemia were admitted during the study period, including 728 patients with ALL and 290 patients with AML. Sixteen children(1.6%) presented with peripheral facial paralysis as the initial symptom, including 7 males and 9 females, with a median age of 5 years (214 years), and a median time from onset of facial paralysis to diagnosis of facial paralysis was 9.5 days(131 days).There were 2 cases （12.5%) of Bcell leukemia, 1 case(6.2%) of Tcell leukemia and 13 cases(81.3%) of M2 myeloid leukemia. All 16 patients had unilateral leukemia, 5（31.2%）were left and 11（68.8%）were right. 15 cases underwent head MR examination, 2 cases showed dural and leptomeningeal thickening and enhancement (including 1 case with suspicious facial nerve injury), 1 case showed leukemia infiltration of medial rectus muscle and orbital segment of optic nerve, and 3 cases showed mastoiditis.Cerebrospinal fluid(CSF) was examined in 15 cases, of which 1 case had CSF minimal residual positive and transient elevation of CSF protein occurred in 1 case. One patient gave up treatment after leukemia diagnosis, and the other 15 patients received chemotherapy. Eight patients remains continuous remission with regular chemotherapy，6 patients received hematopoietic stem cell transplantation, and 1 patient died after relapse due to multiple organ dysfunction during chemotherapy. The symptoms of facial paralysis disappeared in 13 cases within 1 month, and in 2 case within 3 months. The median followup time was 44.5(0.23111) months，the 5year eventfree survival(EFS) was(48.2±13.0)％，and 5year overall survival(OS) was (87.5±8.3)％．The 5year EFS and 5year OS of 13 AML children were (51.3±14.6) % and (84.6±10.0) %, respectively, with 5year OS 100% in the transplant patients and (87.5±11.7) % in the onlychemotherapy patients, showing no statistical difference (P=0.48). Conclusion：Peripheral facial paralysis as the initial symptom of childhood acute leukemia is likely to be misdiagnosed. For children with peripheral facial paralysis, comprehensive examination should be completed to exclude secondary causes before steroid therapy. Intensive central nervous system therapy should be considered in children with acute leukemia with facial paralysis. The overall survival rate of AML children with facial paralysis was not significantly affected, but they were more likely to relapse.

Key words: Peripheral facial paralysis, Children, Leukemia