成年人朗格汉斯细胞组织细胞增生症21例临床病理特征分析

目的 分析成年人朗格汉斯细胞组织细胞增生症(LCH)的临床特征、治疗及预后.方法 对2010年3月至2017年3月南京医科大学第一附属医院及南京市第二医院收治的年龄≥18岁的21例成年LCH患者资料进行回顾性分析,总结其临床表现、实验室检查结果 、治疗方法和预后.结果21例LCH患者中男性14例,女性7例,中位年龄43岁(22~62岁).4例为单系统、单病灶,5例为单系统、多病灶(Hand-Schuller-Christian病),12例为多系统、多病灶(Letter-Siwe病);其中7例有重要器官受累(肺部3例、骨髓2例、肝脏1例、脾脏1例),为高风险患者.所有患者中位生存时间为36个月(1~89个月),其中9例单系统病变及5例多系统病变伴低风险患者均生存,病情稳定,未出现疾病复发,生存时间为4~89个月;7例多系统病变伴高风险患者中3例生存,病情稳定,未复发,4例死亡.结论 成年人LCH发病率较低,患者中男性居多,与儿童LCH不同,成年人LCH临床多为多病灶、多系统病变.多系统病变伴高风险患者治疗反应差,需探索新型治疗方案.

Clinicopathological analysis of 21 patients with adult langerhans cell histiocytosis

Objective To observe the clinical characteristics, treatment and prognosis of adult patients with langerhans cell histiocytosis (LCH). Methods The clinical data of 21 adult patients with LCH≥18 years old from March 2010 to March 2017 in the Second Affiliated Hospital of Southeast University and the First Affiliated Hospital with Nanjing Medical University were retrospectively analyzed, and the clinical manifestations, laboratory tests, treatments and prognosis were observed. Results A total of 21 patients included 14 male cases and 7 female cases. The median age was 43 years old (22-62 years old). There were 4 patients with single system and single lesion, 5 patients with single system and multi-lesion (Hand-Schuller-Christian) and 12 patients with multisystem and multi-lesion (Letter-Siwe disease). Risk-organ involvement was observed in 7 cases (3 lung cases, 2 bone marrow cases, 1 liver case and 1 spleen case ). The median overall survival time of all patients with LCH was 36 months (1-89 months), including 9 patients with single-system disease and 5 patients with multisystem disease without recurrence and unstable condition (survival time: 4-89 mouths). Among 7 patients with multisystem disease with high-risk organ involvement, 3 survived with no recurrence and 4 died from disease progression. Conclusions The incidence of adult LCH featured by the involvement of more multisystem and multi-lesion in clinic is low, and male cases are in the majority. Patients with multisystem disease and risk organ involvement have poor response to current therapy, and new treatments need to be explored.