徐瑞荣治疗朗格汉斯细胞组织细胞增生症经验  

XU Ruirong’s experience in the treatment of Langerhans cell histiocytosis

作　　者：高畅 吕纯懿 汤欣雨 徐瑞荣[2] GAO Chang

机构地区：[1]山东中医药大学,山东济南250014 [2]山东中医药大学附属医院,山东济南250014

出　　处：《中医临床研究》2024年第19期82-87,共6页Clinical Journal Of Chinese Medicine

基　　金：端粒损耗在骨髓衰竭中发病机制及芪莲益髓清毒颗粒的调控作用(ZR2020KH023)。

摘　　要：朗格汉斯细胞组织细胞增生症是一类病因不明、临床表现多样的罕见病,多见于儿童。其特征在于克隆性病理组织细胞组成的肉芽肿病变。根据临床损害的数量,本病可分为孤立性皮肤和骨骼单系统病变朗格汉斯细胞组织细胞增生症和风险性多系统病变朗格汉斯细胞组织细胞增生症。风险性多系统病变常常累及骨骼、皮肤、造血系统和中枢神经系统以及一些高风险脏器,如肺、肝等。临床表现主要为骨损害、皮疹、肝脾肿大、淋巴结肿大、发热等。治疗以系统化疗为主,风险性多系统病变较孤立性皮肤和骨骼单系统病变化疗效果差,尤其是累及肝和造血功能的患者,病死率较高,且化疗期间易出现一系列不良反应,影响治疗结局。中医认为本病可归为“流皮漏”“虚劳”“骨痹”等范畴。徐瑞荣教授认为本病多因肾阴亏虚、肝脾不调,气郁日久化热、生风生痰所致。他认为,治疗上以滋肾养阴清热、养血祛风化痰为原则,并应用中医膏方这一独特的剂型临证加减,灵活应用风药、动物药标本兼治、攻补兼施,有效改善朗格汉斯细胞组织细胞增生症患者骨髓系统损害以及皮肤系统受累等症状,使患者生活质量明显提高。现介绍徐瑞荣教授运用膏方治疗朗格汉斯细胞组织细胞增生症经验并附验案1则,以飨同道。Langerhans cell histiocytosis(LCH)is a rare disease with unknown etiology and diverse clinical manifestations,which Lis more common in children.It is characterized by granulomatous lesions composed of clonal pathological tissue cells.According to the number of clinical lesions,the disease can be divided into single system LCH(SS-LCH)and multi system LCH(MS-LCH).MS-LCH often involves the skeletal system,skin system,hematopoietic system,central nervous system and some high-risk organs,such as lung and liver.The main clinical manifestations are bone damage,rash,hepatosplenomegaly,lymphadenopathy,fever,etc..The treatment is mainly based on systemic chemotherapy.Compared with SS-LCH,MS-LCH has a relatively poor chemotherapy effect,especially in patients with liver and hematopoietic function disorders.The mortality rate is high,and a series of adverse reactions are prone to occur during chemotherapy,affecting the treatment outcome.Traditional Chinese medicine believes that the disease can be classified as Liupilou(流皮漏),Xulao(虚劳),Gubi(骨痹),and other categories.Professor XU Ruirong believes that the disease is mostly caused by kidney Yin(阴)deficiency,liver and spleen imbalance,Qi(气)stagnation,Wind and phlegm.He believes that the treatment should be based on the principles of nourishing the kidney,nourishing Yin,dispelling wind and resolving phlegm,and the application of the unique dosage form of traditional Chinese medicine ointment would effectively ameliorate the symptoms of LCH patients.This paper introduces Professor XU Ruirong’s experience in the treatment of LCH with ointment combined with one case,in order to provide reference for clinical practice.

关 键 词：朗格汉斯细胞组织细胞增生症 中医膏方 补肾益髓 滋阴清热 

分 类 号：R329.2[医药卫生—人体解剖和组织胚胎学]