淋巴管肌瘤病201例临床特点及中西医结合治疗疗效分析  

Clinical features and effect of integrated traditional Chinese and Western medicine in the treatment of lymphangioleiomyomatosis:an analysis of 201 cases

作　　者：管鑫 吴家良 朱远航 GUAN Xin;WU Jialiang;ZHU Yuanhang(Department of Prevention and Treatment,Xiangshan Hospital of Traditional Chinese Medicine,Huangpu District,Shanghai,Shanghai 200020,China)

机构地区：[1]上海市黄浦区香山中医医院治未病科,上海200020

出　　处：《安徽医药》2025年第5期935-940,共6页Anhui Medical and Pharmaceutical Journal

基　　金：上海市进一步加快中医药传承创新发展三年行动计划[ZY(2021-2023)-0207-02];上海市卫健委中医药科研项目(2024QN091);上海市黄浦区科委科研项目(HZQ202110)。

摘　　要：目的总结淋巴管肌瘤病(LAM)的临床特点及诊疗情况,提高中西医结合治疗LAM疗效,减少漏诊和误诊。方法收集2012年1月至2021年12月于上海市黄浦区香山中医医院LAM中医特色专科门诊就诊并定期随访的201例LAM病人的临床诊治资料,进行分析总结。结果201例LAM病人均为女性,确诊年龄(36.18±8.73)岁,因疾病罕见,误诊率高达39.80%,最常见的误诊疾病为气胸。诊断标准近年发生改变,在诊断过程中正在逐渐减少使用有创操作。与普通人群相比,患有LAM的女性生育能力较差,生育的孩子也较少,妊娠发生早产和流产的数量更多,晚期LAM病人应避免受孕。临床表现最常发生为气胸(48.76%)、肾血管平滑肌脂肪瘤(33.33%)、结节性硬化(7.96%)、乳糜胸(22.40%)、乳糜腹(9.95%)、腹膜后包块(27.9%)、肺动脉高压(18.41%)。存在肺功能损害、气胸和乳糜胸的病人建议服用西罗莫司,上海市黄浦区香山中医医院LAM专病团队在总结经验的基础上,制定了淋巴管肌瘤病中医诊疗方案,认为LAM总的治疗原则为疏肝理气、补肾敛肺,主要方药为四逆散加减,根据LAM病人不同证型进行辨证施治用药,服用中药后可以明显改善气促、咳喘、胸闷等临床症状。结论LAM属于我国发布的罕见病之一,是一种以肺部弥漫性囊性病变为特征的多系统转移性肿瘤疾病,病人的症状取决于受累的器官。因为发病率极低、临床诊断困难、对疾病认识程度不足等原因,LAM的误诊率、漏诊率较高。中医药四逆散加减治疗LAM具有独特优势。Objective To summarize the clinical features,diagnosis and treatment of lymphangioleiomyomatosis(LAM),to improve the curative effect of integrated traditional Chinese and Western medicine in the treatment of LAM,and to reduce missed and misdiagnosed cases.Methods We collected and analyzed the clinical diagnosis and treatment data of 201 LAM patients who were treated in the LAM TCM Specialty Clinic of Xiangshan Hospital of Traditional Chinese Medicine,Huangpu District,Shanghai from January 2012 to December 2021 and followed up regularly.Results The 201 LAM patients were all female,with an average diagnosis age of(36.18±8.73)years,and the misdiagnosis rate was as high as 39.8%due to rareness of the disease,and LAM was most commonly misdiagnosed as pneumothorax.The diagnostic criteria of the disease have changed in recent years,and the use of invasive procedures in the diagnostic process is gradually decreasing.Compared with the general population,women with LAM are less fertile,have fewer children,and have a higher rate of premature birth and miscarriages,and thus pregnancy should be avoided in patients with advanced LAM.The most common clinical manifestations were pneumothorax(48.76%),renal angiomyolipoma(33.33%),tuberous sclerosis(7.96%),chylothorax(22.40%),chyloperitoneum(9.95%),retroperitoneal mass(27.9%),pulmonary hypertension(18.41%).Patients with lung function impairment,pneumothorax and chylothorax were recommended to take sirolimus.On the basis of summarizing experience,the LAM disease specialist team of Xiangshan Hospital of Traditional Chinese Medicine formulated a TCM diagnosis and treatment plan for lymphangioleiomyomatosis,and believed that the overall treatment principle of LAM disease was to invigorate the kidney and restrain the lung,soothe the liver and regulate qi.The main prescription was Sini San.The dosage of medicine was determined according to the syndrome differentiation of LAM patients.After taking the traditional Chinese medicine,clinical symptoms of shortness of breath,cough and asthma,

关 键 词：淋巴管肌瘤病 罕见病 西罗莫司 四逆散 

分 类 号：R730.262[医药卫生—肿瘤] R73[医药卫生—临床医学]