35例朗格罕细胞组织细胞增生症的临床病理影像特点、预后及治疗方法分析

Analysis of Clinical,Iconographical and Pathological Characteristics,Prognosis and Treatment Methods for 35 Cases of Langerhans Cell Histiocytosis

本研究目的分析朗格罕细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)的临床病理特点及治疗方法,提高对该病的诊治水平。对35例LCH病例进行回顾性分析,按照年龄分为未成年组(<14岁)及成年组(≥14岁),对其临床症状、体征、影像学、病理检查结果、治疗情况及预后进行评估。结果表明:LCH临床特点表现多种多样,错综复杂,单系统受累患者以手术治疗效果较好,多系统受累患者以联合化疗效果较好。经过系统治疗后,中位随访时间为3年,1年总生存率(OS)为94%±4%,2年OS为91%±5%,3年OS为86%±7%。未成年组与成年组3年OS分别为94%±6%和81%±10%,未成年组OS优于成年组,但是由于病例数较少差异无统计学意义。结论:LCH是一种容易误诊的疾病,年龄为预后的主要影响因素。病理活检是其确诊的金标准,PET-CT对该疾病明确分期及病变范围有很大意义。成年LCH患者肺部容易受累及,联合化疗能改善患者的预后,可根据病变的分期及分组选择恰当的治疗方法。

Purpose of this study was to analyse the characteristics of clinical, iconographical, pathological and treatment methods of Langerhans cell histiocytosis （ LCH）, so as to improve the diagnosis and treatment level of this disease. The clinical datas of 35 LCH patients were studied retrospectively. These patients were divided into 2 groups according to age 〈 14 years old and ≥ 14 years old. The clinical symptoms were analysed and the signs, imageology and pathology manifestation and treatment results were evaluated. The results showed that LCH clinical manifestations were diverse and complex. Surgical treatment for patients with single system involvement of LCH was better than that of multi-system involvement of LCH （MS-LCH）. For the latter, combined chemotherapy effects was better. After 3-year follow-up, 1-year OS was 94%±4%, 2-years OS was 91%±5%, 3-year OS was 86% ± 7%. 3 years OS of group 〈 14 years old and 14 years old was 94% ±6% and 81% ±10% respectively. The OS of former was better than that of the later, but because a small number of cases, this difference was not statistically significant. It is concluded that LCH is easy to be misdiagnosed, the pathological biopsy is the gold standard of LCH diagnosis. The PET-CT can be of great help in identifying stages and finding lesion areas of the disease. Pulmonary Langerhans cell histiocytosis （PLCH） is more common in adult. Combined chemotherapy can improve the prognosis of the patients. The treatment methods should be choosed according to the stage and classification of disease.