儿童朗格汉斯细胞组织细胞增生症26例临床分析

Analysis of 26 children with Langerhans cell histiocytosis

目的提高对儿童朗格汉斯细胞组织细胞增生症(LCH)临床特点的认识,探讨应用改良LCH-Ⅲ方案治疗的有效性和安全性。方法 26例LCH患儿均在确诊前完善必要的实验室检查、皮疹印片或组织病理检查,按Lavin-Osband法进行分级和分型。轻型者仅行局部治疗,其余病例共分为3组,分别按照改良LCH-Ⅲ方案进行化疗。结果 LCH病变可累及多个系统和脏器,可伴有不同程度功能障碍。26例患儿中,4例采用局部治疗,22例采用改良LCH-Ⅲ方案治疗(其中1例先采用局部治疗,病情进展再用此方案),6周时总有效率为72.7%,18个月时总有效率为86.4%。中位随访期36个月(24～50个月),治愈15例,稳定6例,进展或恶化3例,死亡2例。结论 LCH确诊时临床表现多样,病情轻重不一。改良LCH-Ⅲ方案可针对LCH临床分型和分级进行分层治疗,疗效满意,不良反应及治疗相关并发症少,值得临床推广应用。

Objective To improve the knowledge of clinical features of Langerhans cell histiocytosis （LCH） in children, and to explore the curative effect and safety in treating LCH by using of modified LCH-Ⅲ protocol. Methods Twenty-six （26） cases were diagnosed as LCH based on clinical manifestations, laboratory tests, and bone or skin lesion biopsies. All the cases were staged and scored with Lavin-Osband criteria. Mild cases were treated with localized operation only, other cases were divided into three groups, and applied with modified LCH-Ⅲ protocol. Results LCH lesions could lead to damage to most organs or system functions. Among all the 26 cases, 4 adopted surgery （1 changed later to modified LCH-Ⅲ protocol as conditions worsened）. The total curative effect rate was 72.7% at 6 weeks, 83.4% at 18 months. Medium follow-up time was 36 months （24 months to 50 months）. Fifteen cases were cured. The condition of 6 cases was stable and 3 cases have worsened. Two patients died. Conclusions Children with LCH have diversed manifestations ranged from mild to severe degrees. LCH children can be treated with modified LCH-]H protocol based on staging and scores with satisfactory effectiveness and safety.