摘要
目的探讨颅脑朗格汉斯细胞组织细胞增生症(LCH)的临床分型与治疗方法。方法选择中山大学孙逸仙纪念医院神经外科自2008年1月至2019年1月行颅脑手术并且术后病理免疫组化结果符合LCH诊断标准的患者,参考国际组织细胞协会临床分型将其分为7型:Ⅰ型:颅骨单部位,Ⅱ型:颅骨单部位可危及中枢神经系统(CNS),Ⅲ型:颅骨多部位,Ⅳ型:CNS单部位,Ⅴ型:CNS多部位,Ⅵ型:多系统低危,Ⅶ型:多系统高危。根据分型采取2种治疗方法:Ⅰ型采取单纯局部病灶手术切除,Ⅱ~Ⅶ型进行颅脑病灶切除或活检后按日本朗格汉斯细胞组织细胞增生症研究组(JLSG)制定的方案化学治疗,并按JLSG疗效标准进行评价。结果研究共纳入Ⅰ型17例,Ⅱ型2例,Ⅲ型1例,Ⅳ型1例,Ⅴ型0例,Ⅵ型10例,Ⅶ型15例。随访1~10年,Ⅰ型17例患者均未见复发;Ⅱ~Ⅶ型29例患者术后化学治疗中诱导治疗有效27例,诱导有效率为93.1%;2例化学治疗无效改用其他化学治疗方案,其中1例随访1年病情稳定。结论颅脑LCH Ⅰ型患者局部手术切除可达临床治愈,Ⅱ、Ⅲ、Ⅳ、Ⅵ、Ⅶ型患者行病灶活检或切除术后联合JLSG方案化学治疗能够获得较好疗效,对初始诱导6周无反应和疾病进展的LCH患者及时更换挽救方案能改善疗效。
Objective To explore the clinical classifications and treatments of craniocerebral Langerhans cell histiocytosis(LCH).Methods Patients with histopathologically conformed LCH in our hospital from January 2008 to January 2019 were classified into 7 types according to the International Histocell Association clinical typing:type Ⅰ(single site of skull),type Ⅱ(single site of skull and involvement of central nervous system[CNS]),type Ⅲ(multiple sites of skull),type Ⅳ(single site of CNS),type V(multiple sites of CNS),type Ⅵ(low-risk multiple sites of multisystem),and type Ⅶ(high-risk multiple sites of multisystem).According to the classification,two treatment methods were adopted.Group A(patients with type Ⅰ)was treated with local resection,and Group B(patients with type Ⅱ,Ⅲ,Ⅳ,Ⅵ,and Ⅶ)was treated with chemotherapy based on Japanese Langerhans Cell Histiocytosis Study Group(JLSG)protocol after local resection or biopsy;according to the efficacy standards of JLSG,the treatment efficacies were evaluated.Results The study covered 17 patients from Group A(type Ⅰ)and 29 patients from Group B,including 2 with type n,one with type ID,one with type Ⅳ,10 with type Ⅵ,and 15 with type Ⅶ.During the follow-up period of one-10 years,there was no recurrence in 17 patients from group A;27 out of 29 patients from group B had effective induction in postoperative JLSG chemotherapy,enjoying induction efficiency of 93.1%;two patients were treated with other chemotherapy regimes,and one of them was followed up for one year and his condition was stable.Conclusion For type Ⅰ craniocerebral LCH patients,clinical cure can be obtained with local resection;for type Ⅱ,Ⅲ,Ⅳ,Ⅵ,and Ⅶ LCH patients,good curative effect can be obtained with JLSG chemotherapy after local resection or biopsy;for LCH patients who have no reaction or disease progress in the first 6 weeks of induction,the curative effect can be improved by changing the rescue plan in time.
作者
彭岸雄
刘正豪
郑眉光
雷炳喜
邓跃飞
Peng Anxiong;Liu Zhenghao;Zheng Meiguang;Lei Bingxi;Deng Yuefei(Department of Neurosurgery,Sun Yat-sen Memorial Hospital of Sun Yat-sen University,Guangzhou 510120,China)
出处
《中华神经医学杂志》
CAS
CSCD
北大核心
2020年第7期706-710,共5页
Chinese Journal of Neuromedicine
